July - September, 2004

Journal of Pakistan Association of
Dermatologists
Volume 1 4, Number 3 July-September, 200 4
Editor
Ijaz Hussain
Associate Editors
Farhana Muzaffar
Zahida Rani
Faria Asad
Advisory Board Editorial Board

Ashfaq Ahmed Khan Ahsan Hameed Nasser Rashid Dar
Atif Hasnain Kazmi Amor Khachemoune Pervaiz Iqbal
Badr S. Dhanani Arfan ul Bari Shahbaz A. Janjua
Hasina Thawerani Muhammad Arif Maan Shahbaz Aman
Iqbal Chowdhry Ghulam Mujtaba Tahir Anees
Iqbal Tareen Ian MacColl Tahir Jamil Ahmad
Khadimullah Kakakhel Jameel A. Shaheen Tariq Rashid
Muhammad Jahangir Khalid Hussain Tariq Zaman
Naeem Iqbal Khawar Khurshid Yasmeena Khan
S. M. Azam Bokhari Muhammad Khalid Zarnaz Wahid
S.M. Shamim Naseema Kapadia Zohra Zaidi
Sabrina Suhail Pal
Simeen Ber Rahman
Tahir Saeed Haroon
Publication Manager
Mr. Omar Abdul Aziz
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 2003 Any material published in JPAD is copyright of Pakistan Association of
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Journal of Pakistan Association of
Dermatologists
Volume 1 4, Number 3 July-September, 200 4
Contents
Editorial
The guardian of the genome: p53 107
Shahbaz A. Janjua
Original articles
Cutaneous manifestations of systemic lupus erythematosus in Pakistani children 110
Farhana Muzaffar
Cutaneous leishmaniasis in Sadda, Kurram Agency, Pakistan 114

Sahibzada Mahmood Noor, Dildar Hussain
Olive oil: an effective emollient for lichen simplex chronicus 118

Syed Muhammad Shamim, Kishwar Sultana, Fareeda Islam, S.I Ahmed
Family history of psoriasis and recent infectious disease are risk factors for the first episode of
acute guttate psoriasis 124
Shahzana Naqqash , Tameez-ud-deen, Shahid Naqqash, Abdul Quddus Butt
Review articles
Porokeratosis : A review of unique group of keratinizing disorders 130

Arfan ul Bari, Simeen Ber Rahman
Management of atopic dermatitis: a review 140

Amer Ejaz, Naeem Raza
Stat corner
Biostatistics – I: Introduction, role and application in medicine 148
Tariq Zaman, Abbas Raza
Case reports
Dyskeratosis congenita in a Saudi boy: an uncommon genodermatosis 153
A. Y. Saadeldin, Satti A. Satti, Ali S. Dammas
Nevoid psoriasis: an uncommon blaschkolinear dermatosis 157
Arfan ul Bari, Simeen Ber Rahman
Quiz
Generalized pustular rash of acute onset 161
Asher Ahmed Mashood
News 164
Information for authors 165

Journal of Pakistan Association of Dermatologists 2004; 14: 107-9.
Editorial
The guardian of genome: p53
Shahbaz A. Janjua
Ayza Skin and Research Center, Lalamusa, Pakistan
Cancer is essentially a genetic disorder. So cell death. Both copies of the tumor
far, more than one hundred cancer related suppressor gene must be inactivated for
genes have been discovered, several of complete loss of function unlike oncogenes.
which are implicated in the natural history One group of tumor suppressor genes
of cancer because they have constantly been restricts cellular growth by inhibiting the
found mutated. 1 The mutations could be cell cycle and cell division, down-regulating
inherited or acquired. Inherited mutations growth signals, or promoting cell death
that predispose individuals to cancer while the second group does not directly
formation are termed germline, while participate in growth regulation, but rather
acquired mutations that contribute to tumor maintains the integrity of the human
development are known as somatic. genome.
Mutations that occur in critical grow th
regulatory genes resulting in variations in The p53 tumor-suppressor gene is the most
cellular proliferation and survival, striking example that is mutated in about
subsequently contribute to the selection of half of almost all types of cancer arising
dominant tumor populations. from a wide spectrum of tissues.1 The p53
gene is located on the long arm of
Oncogenes and tumor suppressor genes chromosome 17 and contains 11 exons
make two broad classes that become spanning some 20000 bp of genomic
mutated contributing to cancer formation. sequence. The gene encodes a 53 kd nuclear
Cancer-promoting genes or oncogenes were phosphoprotein of 393 amino acid residues
originally identified as viral genes that which is well known as p53 protein. The
"transform" a normal cell into a malignant p53, also termed as guardian of the genome
cell. Normal counterparts to these viral (as it protects DNA integrity in response to
oncogenes in the human genome, well cytotoxic stress, including radiation), was
known as proto-oncogenes, have been discovered in the late 1970s.2,3 It has been
detected, most of which function as growth- implicated in the control of the cell cycle,
signaling molecules that become mutated DNA repair and synthesis, cell
and are perpetually "turned on”. Tumor differentiation, genomic plasticity, and
suppressor genes, on the other hand, programmed cell death. 4,5 The activity of the
negatively regulate cell growth or promote p53 tumor-suppressor protein has a key role
in controlling both cancer and aging. It
Address for Correspondence
Dr. Shahbaz A. Janjua, MD, would be pertinent to note here that
Ayza Skin and Research Center, underactivity of p53 encourages the growth
Lalamusa, Pakistan
of cancer, and overactivity can accelerate
Email: dr_janjua@yahoo.com
the aging process.
107
The guardian of genome: p53 Shahbaz A. Janjua
The p53 protein which is synthesized after The correlation between the incidence of
the infliction of DNA damage, functions to squamous cell carcinoma and mutations in
protect the cells from malignant p53 tumor suppressor genes has been well
transformation by causing cell-cycle arrest characterized. 10 The chief risk factor for
at the G1 phase until the DNA damage has squamous-cell carcinoma, is exposure to
been repaired. Once the damage is repaired, ultraviolet light which is highly mutagenic,
p53 is degraded. As mentioned earlier, a partly as a consequence of the characteristic
loss of this protective influence occurs in pyrimidine dimer premutagenic lesions it
approximately 50% of human tumors in generates in DNA. 11 Of all the
which p53 is inactivated by a mutation in its experimentally examined mutagens,
gene or by the binding of proteins encoded ultraviolet radiation leaves the most
by viral or cellular oncogenes.6 The distinctive fingerprint in DNA: unrepaired
mutations result in reduced binding of the cytosine dimers induce tandem mutations, in
p53 with the damaged DNA and subsequent which two adjacent cytosine residues
accumulation of mutated p53 in the cell (cytosine-cytosine) are replaced by two
nuclei of the affected cells to the extent that thymine bases (thymine-thymine), an event
it becomes detectable by routine that occurs very rarely unless there is
immunocytochemistry. 7 This makes it the exposure to ultraviolet radiation. Three of
most frequently inactivated protein in the first 15 mutations discovered in the p53
human cancer and therefore an important gene of squamous-cell carcinomas of the
pathway to target for cancer therapy. In skin were just such tandem substitutions,
addition to representing the most common directly incriminating both exposure to
genetic defects in human cancer, the ultraviolet light as the cause of damage to
spectrum of p53 mutations has characteristic the p53 gene and the loss of tumor-
fingerprints that can be correlated with the suppressor function in the development of
DNA damage specific to certain definitive the cancers.10
causes of cancer (e.g. UV-B radiation,
aflatoxin, and oxidative processes).8 It would be interesting to note that p53 is not
an oncogene and the mutated forms may not
The mutated forms of p53 may also interact necessarily result in an oncogenic process. It
with different sets of transcription sites, was evident from the description of p53
resulting in increased proliferation of cells mutations in at least two nonmalignant
because p53 is also a transcription factor.9 hyperproliferative processes including
Mutations in the p53 gene have been keloid and rheumatoid arthritis.12,13
observed in many actinic keratoses, basal
cell carcinomas, and squamous cell It has also been hypothesized that p53
carcinomas, and in a small proportion of mutations predispose cells to
malignant melanomas. Specific types of hyperproliferation, resulting in keloid
pyrimidine transitions have pointed to a role formation because p53 mutations have been
for UV light in these mutations.7 noted in the keloid fibroblasts.12
108
The most challenging task is the 5. Hollstein M, Sidransky D, Vogelstein

B, Harris CC. p53 Mutations in human
development of drugs to mimic p53 tumor-
cancers. Science 1991; 253: 49-53.
suppressor function that is being aided by 6. Greenblatt MS, Bennett WP, Hollstein
rapid advances in studies of p53 molecular M, Harris CC. Mutations in p53 tumor
mechanisms. The failure of chemotherapy suppression gene: clues to cancer
etiology and molecular pathogenesis.
and resistance to radiotherapy has been Cancer Res. 1994; 54: 4855-78.
attributed to multidrug-resistance gene, 7. McNutt NS, Saenz-Santamaria C,
MDR1, which confers cross-resistance to Volkenandt M et al. Abnormalities of
p53 protein expression in cutaneous
hydrophobic natural-product cytotoxic drugs disorders. Arch Dermatol 1994; 130:
making that treatment ineffective. It has 225-32.
been demonstrated that the expression of 8. Gasparro FP. p53 in dermatology. Arch
Dermatol 1998; 134: 1029-32.
MDR1 is up-regulated by certain mutants of 9. Dittmer D, Pati S, Zambetti G et al.
p53.9,14 Then the role of normal functioning Gain of function mutations in p53. Nat
p53 in allowing time for the repair of Genet 1993; 4: 42-6.
10. Brash DE, Rudolph JA, Simon JA et al.
radiation-induced DNA damage during the A role for sunlight in skin cancer: UV-
G1 phase of the cell cycle, suggests that the induced p53 mutations in squamous cell
response to radiotherapy or chemotherapy carcinoma. Proc Natl Acad Sci U S A
1991; 88: 10124-8.
may depend in part on the status of p53 in 11. Drobetsky EA, Grosovsky AJ,
tumors before treatment. A full clinical Glickman BW. The specificity of UV-
analysis of various types of tumors still induced mutations at an endogenous
locus in mammalian cells. Proc Natl
remains to be explored to learn whether Acad Sci U S A 1987; 84: 9103-7.
treatment is more or less successful, 12. Saed GM, Ladin D, Olson J et al.
depending on the type of p53 alteration in Analysis of p53 gene mutations in
keloids using polymerase chain
the primary tumor, and whether different
reaction-based single-strand
treatments and treatment schedules should conformational polymorphism and
then be selected on the basis of the DNA sequencing. Arch Dermatol 1998;
134: 963-7.
involvement of p53.
13. Firestein GS, Nguyen K, Aupperle KR
et al Apoptosis in rheumatoid arthritis:
References p53 overexpression in rheumatoid
arthritis synovium. Am J Pathol 1996;
1. Harris CC, Hollstein M. Clinical 149: 2143-51.
implications of the p53 tumor- 14. Chin KV, Ueda K, Pastan I, Gottesman
suppressor gene. N Engl J Med 1993; MM. Modulation of activity of the
329: 1318-27. promoter of the human MDR1 gene by
2. Lane DP, Crawford LV. T antigen is Ras and p53. Science 1992; 255: 459-
bound to a host protein in SV40- 62.
transformed cells. Nature 1979; 278:
261-3.
3. Linzer DI, Levine AJ. Characterization
of a 54K dalton cellular SV40 tumor
antigen present in SV40-transformed
cells and uninfected embryonal
carcinoma cells. Cell 1979; 17: 43-52.
4. Levine AJ, Momand J, Finlay CA. The
p53 tumour suppressor gene. Nature
1991; 351: 453-6.
109
Cutaneous manifestations of SLE in Pakistani children Farhana Muzaffar
Original article
Cutaneous manifestations of systemic
lupus erythematosus in Pakistani children
Farhana Muzaffar
Department of Dermatology, Institute of Child Health/Children Hospital, Lahore.
Abstract Background Systemic lupus erythematosus (SLE) is a multisystem disease of autoimmune

etiology. Cutaneous changes are seen in more than two -third of patients.
Objectives The present study was planned to evaluate cutaneous manifes tations of SLE in
children.
Patients and methods Fifteen cases of SLE were collected from the Department of Pediatric
Dermatology, Children Hospital, Lahore. The diagnosis was based on American Rheumatism
Association criteria. Cutaneous changes were recorded on a predevised pro forma.
Results Age of onset was 5-13 years in 14 (93.3%) children. One case of neonatal LE was
seen. There were 8 (53.3%) females and 7 (46.7%) males. Malar rash was present in 10
(66.6%), photosensitivity in 8 (53.3%), diffuse hair loss in 6 (40%), hyperpigmentation in 5
(33.3%), vascular lesions in 5 (33.3%), mucosal lesions in 3 (20%), nail changes in 2
(13.3%), bullous lesions in 1 (6.7%), livedo reticularis in 1 (6.7%), and rheumatoid nodules
in 1 (6.7%). The single case of NLE had generalized scaly lesions.
Conclusion Cutaneous changes in children are different from those seen in adults. Female
preponderance was not seen in children. Photosensitivity and vascular lesions were less
frequent while the discoid rash was rare. Peripheral gangrene, chilblain and Raynaud’s
phenomenon were not seen. Neonatal LE was a rare entity.
Key words SLE, neonatal LE, cutaneous manifestations.
Introduction Caucasian children with a male to female

ratio of 1:4.5 between the ages of 6 and 18
Systemic lupus erythematosus (SLE) is a
years. 4 It is reported that 15-17% of all
multisystem disease of autoimmune
patients with SLE, the disease starts before
etiology. Integument is affected in more
than two-third of cases.1-3 Cutaneous 16 years. Rarely the babies born to
mothers with lupus may develop LE called
changes constitute four of 11 criteria of
neonatal lupus erythematosus (NLE), due
American Rheumatism Association
to passive transfer of maternal
(ARA) for the diagnosis of SLE. The
disease usually affects adult females. autoantibodies to fetus . Cutaneous
changes of all types occur in 60-90% of
children at any time during the course of
Childhood SLE is a rare entity with an
illness whereas these are seen in 60-78%
incidence of 10-20 in 100000 white
of cases at the time of diagnosis.4 Hence,
Dr. Farhana Muzaffar, the recognition of cutaneous changes of
Assistant Professor of Dermatology, SLE is very important in the early
The Institute o f Child Health/Children’s diagnosis of this potentially dangerous
Hospital, Lahore
110
disease which, otherwise may be missed Table 1 Frequency of different LE-s pecific
by an unwary physician. lesions in 15 case of SLE.
Lesions n (%)
Malar rash 10 (66.7)
Many local studies 5-7 address the subject in Photosensitivity 8 (53.3)
adult patients with SLE; however, scanty Psoriasiform lesions of 1 (6.7)
subacute LE
data are available on the topic in children
of Pakistani origin. The present study was Table 2 Dis tribution of different LE-
conducted to evaluate the cutaneous nonspecific lesions
Lesions n (%)
manifestations of SLE in Pakistani Vascular lesions
children. Telangiectasia 5 (33.3)
Microinfarcts 2 (13.3)
Patients and methods Purpura 2 (13.3)
Chronic ulcers 2 (13.3)
Bullous Erythema multiforme 1 (6.7)
This descriptive study was conducted at Livedo reticularis 1 (6.7)
the Department of Pediatric Dermatology,
Pigmentary changes
Institute of Child Health/Children Hyperpigmentation 5 (33.3)
Hospital, Lahore, from 1st January, 2003 to Hypopigmentation 1 (6.7)
31st December, 2003. Fifteen children of
SLE (diagnosed on the basis of ARA Hair changes
Diffuse alopecia 6 (40)
criteria) were enrolled in the study. A Lupus hair 1 (6.7)
detailed history and physical examination
were recorded. The relevant Others
Ragged cuticle 2 (13.3)
hematological, biochemical and Ichthyosis 2 (13.3)
immunological profile, radiological work- Sclerodactyly 1 (6.7)
up and skin biopsy were carried out. Erythematous papules 1 (6.7)
Rheumatoid nodules 1 (6.7)
Cutaneous changes were further
subdivided as specific (histopathological Table 3 Mucosal sites affected (n=15)
changes characteristic of LE) and non Mucosa e affected n (%)
Lips 3 (20)
specific. Palate 3 (20)
Buccal mucosa 3 (20)
Results Genital mucosa 2 (13.3)
During the one-year period, 15 patients Chronic discoid lesions were not seen in
were diagnosed, 14 children had SLE and any case.
one had neonatal LE. There were 8 female
(53.3%) and 7 (46.7%) male children, with The single case of NLE had generalized
male to female of 1:1.1. Age of onset psoriasiform eruption (Figure 3). She was
ranged from 5-13 years in 14 children with the first baby of a 24-year-old mother who
SLE. The frequency of LE-specific lesions was found to be anti-Ro positive. She had
and LE-nonspecific lesions is shown in fever, skin rash, hepatosplenomegaly and
Table 1 and 2, respectively. cardiac disease. Histopathology from the
back showed changes compatible with LE.
LE-specific included malar rash (Figure The baby was also anti-Ro positive.
1), mucosal ulcers (Figure 2) and
photosensitivity. Oral mucosa was affected Amongst nonspecific lesions vascular
in 3 and genital in 2 patients (Table 3). manifestations were the most frequent
111
Cutaneous manifestations of SLE in Pakistani children Farhana Muzaffar
Figure 2 Ulcers over lips and buccal mucosa.

Face and neck is als o affected.
Figure 1 Malar rash, photosensitive

generalized maculopapular eruption and non-
cicatricial alopecia in a child with SLE.
Figure 4 Bilateral purpuric eruption on the

legs of a girl. Mild scaling is also evident.
previous reports give a female

preponderance. 4
Figure 3 Generalized psoriasiform eruption in
the female infant with neonatal LE. Malar rash was the most frequent finding.
This is in accordance with the previous
(Figure 4). The frequency of other types reports.4,8,9 Malar rash is seen in 30-80% of
of lesions is shown in Table 2. Peripheral patients and it is present in 22-60% of
gangrene, chronic ulcer, chilblain, and cases at the time of diagnosis.4 The
Raynaud’s phenomenon were not seen. incidence of malar rash increases with
disease progression. 9 Photosensitivity was
Discussion rare in our series. Similarly, none of our
patients had discoid lesions. Table 4
The present study affirms the high depicts the comparison of cutaneous
prevalence of cutaneous changes in SLE changes in our patients with that by Font
patients. However, the frequency was et al.8 The difference between two can be
100% in our series than previously due to different racial background.
reported 4 since all of our cases were
collected from the dermatology out-patient Psoriasiform eruption seen in the single
clinic. Secondly, a diverse spectrum of patient of NLE was in consonance with
cutaneous changes can be seen. We literature. 4 This signifies that a
noticed an equal sex ratio whereas psoriasiform eruption in an infant in the
112
Table 4 Comparis on with study in children.

Lesions Present Font et References
study al. [4] 1. Cervera R, Khamashta MA, Font J et
(2003) Spain, al. Systemic lupus erythematosus:
(n=15) 2004 clinical and immunologic patterns of
(n=34) disease expression in a cohort of 1000
Malar rash 66.7 44% patients. Medicine 1993; 72: 113-24.
Photosensitivity 53.3% 23% 2. Costallat LT, Coimbra AM. Systemic
Oral ulcers 20% 9% lupus erythematosus : clinical and
Psoriasiform lesions of 6.7% - laboratory aspects to age at onset.
SCLE Clin Exp Rheumatol 1994; 12: 603-7.
Livedo reticularis 6.7% 3% 3. Tucker LB, Menon S, Miller LC et al.
Adult and childhood-onset systemic
lupus erythematosus: a comparison of
Table 5 Comparis on with study in adults.
onset, clinical features, serology, and
Lesions Present Rabbani outcome. Br J Rheumatol 1995; 34:
study et al. [5] 866-72.
(2003) Pakistan, 4. Silverman ED, Eddy A. Systemic
(n=15) 2003 lupus erythematosus in childhood and
(n=34) adolescence. In: Maddison P,
Malar rash 66.7 31% Isenberg D, Woo P, Glass D, eds.
Photosensitivity 6.7% 33% Oxford Textbook of Rheumatology.
Vascular lesions 33.3% 20% Oxford: Oxford University Press;
Mucosal lesions 20% 20% 1993. p. 756-71.
Pigmentary changes 33.3% 22% 5. Kapadia N. Cutaneous changes in
Dis coid rash - 15% systemic lupus erythematosus
(dissertation). Karachi: College of
presence of systemic features should not Physicians and Surgeons; 1994.
be taken lightly. 6. Ahmed TA, Ikram N, Hussain T et al.
Clinical and laboratory features of
SLE in Pa kistani patients. J Pak Med
Table 5 compares the cutaneous changes Assoc 2002; 52: 12-5.
in children of our series with those in 7. Rabbani MA, Shah SM, Ahmed A.
Cutaneous manifestations of systemic
Pakistani adults. In comparison to adults, lupus erythematosus in Pakistani
malar rash, photosensitivity and vascular patients. J Pak Med Assoc 2003: 53:
lesions were more frequent whereas 539-41.
8. Font J, Cervera R, Espinosa G et al.
discoid lesions are seen more frequently. Systemic lupus erythematosus (SLE)
in childhood: analysis of clinical and
Our results conclude that cutaneous immunologic findings in 34 patients
and comparison with SLE
changes in children are different from characteristics in adults. Ann Rheum
those seen in adults. Female Dis 1998; 58: 456-9.
preponderance is not seen in children. 9. Rood MJ, ten Cate R, van Suijlekom-
Smit LW et al. Childhood-onset SLE:
Photosensitivity is less frequent. Discoid clinical presentation and prognosis in
rash is rare. Peripheral gangrene, chronic 31 patients. Scand J Rheumatol 1999;
ulcer, chilblain, and Raynaud’s 28: 222 -6.
phenomenon were not seen. Neonatal LE
is a rare entity.
113
Cutaneous leishmaniasis in Sadda…. Sahibzada M. Noor and Dildar Hussain
Original article
Cutaneous leishmaniasis in Sadda, Kurram
Agency, Pakistan
Sahibzada Mahmood Noor, Dildar Hussain
Tehsil Headquarter Hospital, Sadda, Parachinar
Abstract Background Cutaneous leishmaniasis (CL) is endemic in the tribal belt bordering
Afghanistan .This study was carried out to determine the demographic and clinical pattern of
the disease in Sadda , Kurram Agency.
Patients and methods CL patients presenting to leishmaniasis clinic of Tehsil Headquarter

Hospital, Sadda from 1st November, 2003 to 30thMarch, 2004 were included in the study. The
patients were diagnosed clinically and confirmed by laboratory confirmation of parasites in a
Giemsa-stained smear prepared from the lesion. All important clinical details were recorded
on a specially designed proforma and patients were registered for the purpose of treatment
and a card was issued to them for subsequent visits and follow up.
Results A total of one hundred and fifty patients with 325 lesions were seen during a period
of five months. Dry type of cutaneous leishmaniasis was seen in 120 (80%) patients and wet
type was noted in 30 (20%) patients. Most of the lesions (98%) were present on exposed parts
of the body. Sixty (40%) patients had one and 75 (50%) had two lesions. More than two
lesions were seen in 10% of patients. Eighty per cent of sufferers were less than 30 years of
age. The disease was more common in males (70%). Family history was positive in 45 (30%)
patients. History regarding traveling to Afghanistan was negative in most of the patients
(98%).
Conclusion CL is endemic in this part of tribal belt. Both type of CL is prevalent among the
local p opulation.
Key words
Cutaneous leishmaniasis, Sadda
Introduction and Chakwal districts in the province of

Punjab.3 In NWFP, the disease has been
Cutaneous leishmaniasis (CL) is a chronic reported from District Dir, 4 Kohat, 5 and
granulomatous infection of Afghan refugees settlements. In
reticuloendothelial cells of skin caused by Afghanistan and Pakistan two Leishmania
the protozoan parasite Leishmania. 1 species; L. tropica causing dry type of
Although, the infection occurs in all lesions and L. major producing wet type of
continents, it is endemic in tropical and lesions are mainly seen. 6 Visceral
subtropical countries. In Pakistan, the leishmaniasis has been reported from
disease is endemic in Sindh and district Dir in NWFP.7
Baluchistan provinces.2 It has also been
reported from Multan, Dera Ghazi Khan Cutaneous leishmaniasis is called saal
dana in Afghanistan and areas of NWFP
Dr. Sahibzada Mahmood Noor,
where it is endemic (saal=year,
32, New Colony, Hango Road, dana=lesion). CL has been common in
Kohat, Pakistan parts of Afghanistan for centuries . 6 During
Email: drnoorsm@hotmail.com Soviet occupation of Afghanistan, tribal
114
belt bordering Afghanistan was the home of Leishmania in a Giemsa-stained sm ear

to millions of Afghan refugees, and from the edge of the lesion. Smear
Kurram Agency in Federally administered examination is the earliest and sole
tribal area (FATA) had the largest method to confirm the clinical diagnosis in
population of Afghan refugees. These war- an endemic area. In our study only smear
related population movements and positive cases were included in the study.
environmental destruction have caused a All the cases were registered and a card
large increase in the CL prevalence in was issued for the purpose of treatment
tribal belt bordering Afghanistan. CL and follow -up. Patients were treated
spread to refugee camps in FATA of according to WHO recommendations with
Pakistan and is now transmitted locally in either sodium stib ogluconate or
these areas.8 meglumine antimonate.
Kurram Agency is divided into three parts Results

Upper Kurram, lower Kurram and central
Kurram. Sadda is the headquarter of the A total of 150 patients were seen in the
lower Kurram with a population of half a clinic during a period of 4 months from 1st
million. Due to increase in the prevalence November, 2003 to 30 th March, 2004.
of cutaneous leishmaniasis in Sadda, a There were 105 (70%) males and 45
Leishmaniasis Clinic was established in (30%) females. Although disease was
2001 where patients are diagnosed on the prevalent in all age groups (range 1-60
basis of smear for parasites and treated years), 83% of the patients were less than
with pentavalent antimonial compounds. 30 years of age. Dry type of leishmaniasis
This study was conducted to determine the was seen in 120 (80%) patients while wet
demographic and clinical pattern of type was noted in 30 (20%) patients Table
cutaneous leishmaniasis in patients 1.
presenting to leishmaniasis clinic of Tehsil
head quarter hospital Sadda. The duration of lesion ranged from 4
weeks to 3 months. The lesions were
Patients and methods situated on the exposed parts of the body
face, hands and feet. Face was the
This study was carried out in the commonest site involved and trunk the
Leishmaniasis Clinic of Tehsil headquarter least Table 2. Family history of
hospital (THQ), Sadda, Kurram agency leishmaniasis was positive in 22 patients.
from 1st November 2003 to 30th April, None of the patients reported traveling to
2004. One hundred and fifty patients with Afghanistan. Majority of patie nts were
clinically suspected leishmaniasis local whereas 17 patients were Afghan
presenting to the Leishmanias is Clinic of refugees. The total number of lesions in all
THQ hospital, Sadda, Parachinar were the patients was 325. Sixty patients (40%)
included in the study. Clinical features had one lesion, 75 (50%) had two lesions
including age, gender, nationality, site and and 15 (10%) had more than two lesions.
number of the lesions, family history, The maximum number of lesions i.e. 6
inquiry regarding visit to Afghanistan was seen in brother and sister who were
were recorded on a register. The lesion from Sherinao, a small village next to Pak-
was examined clinically and diagnosis was Afghan border in Afghanistan Table 3.
confirmed by the presence of amastigotes
115
Cutaneous leishmaniasis in Sadda…. Sahibzada M. Noor and Dildar Hussain
Table 1 Age and gender of the study result of massive bombing carried out in
population (n=150) this area that disturbed the ecology.
Age (years) n (%)
Initially the disease was localized to
0-10 30 (20)
Afghan refugees but gradually it involved
11-20 48 (33)
the local population, which was non
21-30 40 (27) immune to the disease as reported from
31-40 11 (7.3) other parts of country. 8
51-60 9 (6)
Table 2 Anatomical distribution of lesions

In the present study, both types of
(n=325) cutaneous leishmaniasis were seen,
Site n (%) although dry type of lesions were more
Face 103 (32) common (80%). In contrast Rab et al. 9
Hands 82 (25)
Forearms 48 (15) reported wet type of lesions from
Feet 58 (17.8) Baluchistan whereas another study from
Legs 26 (8) Multan10 reported dry type of lesions. The
Trunk 7 (2.2)
occurrence of both type of cutaneous
Total 325 (100)
leishmaniasis may be due to presence of L.
Table 3 Frequency of number of lesions
major as well as L. tropica in this region.
(n=325)
Number of lesions n (%)
1 60 (40) Almost all the patients were local except
2 75 (50) two patients who were admitted in the
3 4 (2.7)
4 1 (0.7)
hospital with extensive lesions referred
5 3 (2) from Afghanistan. This signifies that the
6 2 (1.3) disease is locally endemic. Afghan
refugees living in Sadda are provided
health facilities by Project Directorate
Discussion Health for Afghan refugees, having
separate centers for provision of health
Cutaneous leishmaniasis is a world wide facilities to Afghan refugees.
problem and is endemic in Afghanistan for
centuries. It is of clinical importance
because of its chronicity and its potential Most of the patients were from poor
for local destruction and disfigurement. socioeconomic background living in
The disease is commonly known as saal- congested houses having mud-lined walls
dana in parts of Afghanistan and trib al with poor sanitary conditions. Family
areas where it is endemic .8 Sporadic cases history of involvement was positive in
of cutaneous leishmaniasis were seen in 30% of cases.
Afghan refugees living in this area as
evidenced by the presence of healed
lesions in Afghan refugees. The disease Lesions were found mainly on exposed
has been endemic in Afghanistan for a parts of the body because these are easily
long time. 8 The present outbreak in this accessible sites for the sandflies to bite.
part of tribal belt can be linked to large The duration of lesions ranged from 4
scale movement of refugees across the weeks to three months. The disease was
border and environmental destruction as a more common in 11-20 years age group.
116
Smear for Leishman-Donovan bodies is leishmaniasis in Multan. J Pak Med

the sole method available to confirm these Assoc 2001; 50: 279-81.
4. Rahim F, Jamal S, Raziq F et al. An
cases. Patients presenting with unusual outbreak of cutaneous Leishmanias is
lesions with negative smear were referred in a village of District Dir, N.W.F.P. J
to tertiary level hospitals for diagnosis and Postgrad Med Inst 2003 ;17: 85-8.
5. Mashood AM. Diagnostic yield of
management. Unusual clinical variants of various traditional laboratory
cutaneous leishmaniasis have been investigations in the diagnosis of
reported from other endemic areas.11 Due cutaneous leishmaniasis. J Pak Assoc
Dermatol 2004; 14: 59-63.
to lack of facilities and trained personnel, 6. World Health Organization (WHO).
the disease is overdiagnosed and many Report of Committee. The
skin ailments are wrongly treated as Leishmaniasis: World Health Tech
Rep Ser 701, 1984.
leishmaniasis. 7. Rahim F, Rehman F, Ahmed S, Zada
B. Visceral Leishmaniasis in District
Dir, N.W.F.P. J Pak Med Assoc 1998;
Conclusion 48: 162 -4.
8. Rowland M, Munir A, Durrani N et
al. An outbreak of cutaneous
leishmaniasis in an Afghan refugee
Cutaneous leishmaniasis is endemic in this settlement in north-west Pakistan.
part of tribal area bordering Afghanistan. Trans R Soc Trop Med Hyg 1999; 93:
It is for first time that cutaneous 2.
leishmaniasis has been reported from this 9. Rab MA, Azmi FA, Iqbal J et al.
Cutaneous leishmaniasis in
area. The disease can be controlled by Baluchistan: reservoir, host and
elimination of sand flies and improving sandfly vector in Uthal, Lasbela. J
sanitary conditions. Pak Med Assoc 1996; 36: 134-8.
10. Mujtaba G, Khalid M. Cutaneous
Leishmaniasis in Multan, Pakistan.
Int J Dermatol 1998; 37: 843-6.
References 11. Raja KM, Khan AA, Hameed A,
Rahman SB. Unusual clinical variants
1. Kubba R, Gindan YA. Leishmaniasis. of cutaneous leishmaniasis in
Dermatol Clinic 1989; 7 :331-50. Pakistan. Br J Dermatol 1998; 139 :
2. Jaffrany M, Haroon TS. Cutaneous 111-3.
leishmaniasis in Pakistan. Biomedica
1992; 8: 39-44.
3. Ayub S, Mujtaba G, Khalid M, Bhutta
R. Profile of patients of cutaneous
117
Olive oil: an effective emollient for LSC S. M. Shamim et al.
Original article
Olive oil: an effective emollient for lichen
simplex chronicus
Syed Muhammad Shamim, Kishwar Sultana*, Fareeda Islam, S.I Ahmed
Department Pharmacology and Therapeutics, Karachi Medical & Dental College, Karachi
* Department Anatomy, Hamdard College of Medicine & Dentistry, Hamdard University,
Karachi
Department of Pharmacy, Hamdard University, Karachi
Abstract Background Olive belongs to the family of Oleacae. Olive oil is being used increasingly in
different systemic diseases. In dermatology, it is primarily used as a vehicle but it can have
many other potential uses.
Objectives We tested the efficacy of topical olive oil in the treatment of lichen simplex
chronicus.
Patients and methods Forty male and female patients suffering from lichen simplex
chronicus affecting nape of the neck, arms, back of the legs and ankle. Patients were followed
up weekly for four weeks. Pruritus and dryness were scored on a 4-grade scale i.e. none,
slight, moderate and severe at baseline and on each follow up visit.
Results Significant improvement in pruritus and dryness was noticed in all age groups and
both sexes.
Conclusions Olive oil is effective in controlling dryness and pruritus in lichen simplex
chronicus.
Key words Olive oil, emollient, lichen simplex chronicus.
Introduction massaged and it was considered remedy of

haemorrhoids, skin diseases, according to
The plant Olive (Zaitoon in Arabic and Ibn-ul-Qim as mentioned by Ghaznavi. 1,2
Urdu) belongs the family Oleacae. The The olive oil produces freshness, keeps
olive oil is nutritive, emollient, demulcent, alertness, removes the worms from the
laxative, allays the irritation of digestive gut, gives vigour in old age2 It is known
organs and alimentary canal. Olive oil is that Mediterranean diet represents a useful
used for edible purpose, relieves general and effective mean for prevention of
debility and weakness in all age groups. It arteriosclerosis.3 Effect of olive oil on
relieves constipation and is beneficial cardiovascular disease were evaluated and
when used in fistula and anal fissures. It is the findings suggested that polyphenolic
useful to relieve rheumatic pain, paralysis, compounds found in olive oil are endowed
sciatica. It softens the body when with several biologic activities that may
contribute to lower incidence of coronary
Address for Correspondence heart disease in Mediterranean area, 4 It is
Prof. Syed Muhammad Shamim useful in modifying disease activity in
Department Pharmacology and Therapeutics,
Karachi Medical & Dental College, Karachi systemic lupus erythematosus5 and
rheumatoid arthritis . 6
118
In dermatology, olive oil has many uses. Table 1 Subgroups of patients according to
Besides its use as a vehicle in different Group Age (years) n
topical preparations,7 it is also effective in Male patients (n=40)
alopecia, seborrhoea capitis, lustreless 1. A 10-20 10
2. A1 21-30 10
hair, oral aphthae and burns.2 The
3. A2 31-40 10
experimental work showed its potential
4. A3 41-50 10
benefits in psoriasis.8 Olive oil or fish oil Female patients (n=40)
supplementation was also found to 5. B 10-20 10
improve the symptoms of pruritus in 6. B1 21-30 10
patient of chronic renal failure. 9 7. B2 31-40 10
8. B3 41-50 10
Lichen simplex chronicus (LSC) is a
dermatosis in which lichenification, the were compared for statistical significance.
hallmark of disease, occurs without any
known precipitating factor.10 The disease Results
is common world-wide and it usually
affects adults of both sexes. Nape and Tables 2-5 show the effect of olive oil
sides of neck, around ankles, lower legs, therapy on dryness and pruritus in all
thighs, extensor aspects of arms, and subgroups.
genital regions are the usually affected
sites. Topical steroids with and without Effect on dryness
occlusion, intradermal steroid injections Males
are usually used to treat. Group A In this group, before
treatment dryness was scored as moderate
Considering the growing popularity of in 40% of the patients and severe in 60% .
alternative medicine in dermatology world After four weeks , 70% rated as slight and
over, we aimed to test the efficacy and 30% as moderate dryness (p<0.05).
safety of olive oil in the treatment of this
itchy disorder. Group A1 10% of the patients had
moderate dryness while 90% had severe
Patients an d methods dryness before treatment. At week 4, 50%
had slight and 50% had moderate dryness
Forty male and forty female patients, age (p<0.05).
= 10 years, suffering from lichen simplex Group A2 Before treatment 10%
chronicus (diagnosed clinically) were
of the patients had slight, 40% moderate,
collected from authors’ clinics and divided
and 50% scored severe dryness. After 4
into different subgroups according to the
weeks of treatment, 80% rated slight and
age and sex as shown in Table 1. They 20% moderate dryness (p <0.05).
were advised olive oil to apply on the
affected parts (nape of the neck, arms, Group A3 In 41-50 years group,
back of the legs and ankle) three times a before treatment 10% of the patients had
day regularly. Dryness and pruritus were slight, 40% had moderate and 50% had
scored weekly for 4 weeks. The severe dryness. At the end of four weeks,
assessment score was assigned 60% turned to slight while 40% turned to
qualitatively as none, slight, moderate and moderate (p <0.05).
severe. The pre- and post-treatment scores
119
Table 2 Effect of olive oil treatment on dryness according to age group in male (n=40)
Before
therapy After treatment
Age group and severity of One week Two week Three week Four week
clinical signs n (%) n (%) n (%) n (%) n (%) p value
Group A 10-20 years n=10
None - - - - -
Slight - - - 6 (60) 7 (70) <0.05
Moderate 4 (40) 7 (70) 9 (90) 4 (40) 3 (30)
Severe 6 (60) 3 (30) 1 (10) - -
Group A1 21-30 years n=10
None - - - - -
Slight - - 3 (30) 4 (40) 5 (50) <0.05
Moderate 1 (10) 3 (30) 5 (50) 6 (60) 5 (50)
Severe 9 (90) 7 (70) 2 (20)
None - - - - -
Slight 1 (10) 1 (10) 1 (40) 4 (60) 8 (80) <0.05
Moderate 4 (40) 6 (60) 6 (50) 5 (40) 2 (20)
Severe 5 (50) 3 (30) 3 (10) 1 (10)
None - - - - -
Slight 1 (10) 1 (10) 2 (20) 6 (60) 6 (60) <0.05
Moderate 4 (40) 6 (60) 8 (80) 4 (40) 4 (40)
Severe 5 (50) 3 (30)
Table 3 Effect of o live oil treatment on dryness according to age group in females (n=40)
Before After treatment
treatment One week Two week Three week Four week
Age group and severity of
clinical signs n (%) n (%) n (%) n (%) n (%) p value
Group B 10-20 years n=10
None - - - - -
Slight - - - 1 (10) 2 (20) <0.05
Moderate 2 (20) 2 (20) 10 (100) 9 (90) 8 (80)
Severe 8 (80) 8 (80) - -
Group B121-30 years n=10
None - - - - -
Slight - - - - 5 (20) <0.05
Moderate 3 (30) 5 (50) 9 (90) 9 ( 90) 5 (70)
Severe 7 (70) 5 (50) 1 (10) 1 (10) -
Group B2 31-40 years n=10
None - - - - -
Slight 1 (10) 1 (10) 1 (10) 6 (60) 2 (20) <0.05
Moderate 3 (30) 8 (80) 9 (90) 4 (40) 8 (80)
Severe 6 (60) 1 (10) - - -
None - - - - -
Slight - - - 2 (20) 6 (60) <0.05
Moderate 5 (50) 7 (70) 9 (90) 7 (70) 4 (40)
Severe 5 (50) 3 (30) 1 (10) 1 (10) -
120
Females Group A3 10% of the patients had

Group B Before treatment, 20% of slight pruritus , 40% moderate and 50%
the patients had moderate and 80% had had severe. At the end of 4 weeks , 20%
severe dryness. After four weeks of patients were symptom-free, 70% were
therapy, 20% had slight and 80% had left with slight itching and 10% with
moderate signs dryness (p<0.05). moderate itching (p<0.05).
Group B1 30% patients had Females

moderate and 70% had severe dryness Group B Before treatment, the
before treatment whereas at week 4, 20% pruritus was moderate in 20% of the
had slight, 70% had moderate and 10% patients, while 80% had severe symptoms.
had severe dryness (p<0.05). In contrast, at four weeks, 80% had slight
symptom while 20% had moderate itching
Group B2 In this group, at (p<0.05).
baseline 10% patients had slight, 30%
moderate and 60% severe dryness. At Group B1 The symptom of
week 4, 20% had slight while 80% had pruritus was moderate in 40% of patients
moderate signs (p<0.05). and severe in 60%. At week 4, 60% of the
patients had slight symptom while 40%
Group B3 50% of the patients had had moderate (p<0.05).
moderate while 50% had severe dryness.
After four weeks of treatment, 60% had Group B2 In 10% of the patients
slight while 40% remained with moderate itching was slight, in 30% moderate and in
dryness (p<0.05). 60% severe. With four weeks therapy,
10% had no pruritus, 80% left with slight
Effect on pruritus symptoms and 10% with severe symptom
Males (p<0.05).
Group A At baseline, 40% of the
patients suffered from moderate and 60% Group B3 Before start of the
from severe pruritus. At the end of treatment, 50% of patients had moderate
treatment, 70% complained slight and pruritus while other 50% had severe
30% moderate pruritus (p<0.05). symptom. After four weeks, 90% patients
remained with slight itching and 10%
Group A1 (21-30 years) Before remained with moderate symptom
treatment, 10% had moderate and 90% had (p<0.05).
severe itching while at the end of 4 weeks,
50% of the patients had slight and, 50% Safety profile
had moderate pruritus (p<0.05). None of the patient developed any
cutaneous adverse effect.
Group A2 In this group, 10%
patients had slight pruritus before Discussion
treatment, 50% had moderate while 40%
had severe symptom. At the end of 4 Steroids, topical or intralesional, have
weeks , 10% of the patients were symptom been the mainstay of treatment of LSC. No
free while 80% were having slight pruritus doubt being highly effective, steroids have
and 10% moderate symptom (p<0.05). many inherent, cutaneous and systemic,
adverse
121
Table 4 Effect of olive oil therapy on pruritus) according to age group in male (n=40)
Age group and Before After treatment
severity of clinical Treatment One week Two week Three week Four week
signs n (%) n (%) n (%) n (%) n (%) p value
Group A 10-20 years n=10
None - - - - -
Slight - - - 7 (70) <0.05
Moderate 4 (40) 7 (70) 9 (90) 10 (100) 3 (30)
Severe 6 (60) 3 (30) 1 (10) - -

None - - - - -
Slight - - 3 (30) 4 (40) 5 (50) <0.05
Moderate 1 (10) 3 (30) 6 (60) 6 (60) 5 (50)
Severe 9 (90) 7 (70) 1 (10)

None - - - - 1 (10)
Slight 1 (10) 1 (10) 4 (40) 6 (60) 8 (80) <0.05
Moderate 5 (50) 6 (60) 5 (50) 4 (40) 2 (10)
Severe 4 (40) 3 (30) 1 (10)

None - - - 1 (10) 2 (20)
Slight 1 (10) 1 (10) 2 (20) 5 (50) 7 (70) <0.05
Moderate 4 (40) 6 (60) 8 (80) 4 (40) 1 (10)
Severe 5 (50) 3 (30)
Table 5 Effect of olive oil therapy on p ruritus of the skin according to age group in females (n=40)
Before After treatment
treatment One week Two week Three week Four week
Age group and severity of
clinical s igns n (%) n (%) n (%) n (%) n (%) p value
Group B 10-20 years n=10

None - - - - -
Slight - - 4 (40) 7 (70) 8 (80) <0.05
Moderate 2 (20) 2 (20) 6 (60) 3 (30) 2 (20)
Severe 8 (80) 8 (80) - - -
None - - - - -
Slight - 2 (20) 2 (20) 5 (50) 6 (60) <0.05
Moderate 4 (40) 4 (40) 8 (80) 5 (50) 4 (40)
Severe 6 (60) 4 (40) - - -
None - - - - 1 (10)
Slight 1 (10) 1 (10) 5 (50) 9 (90) 8 (80) <0.05
Moderate 3 (30) 9 (90) 5 (50) 1 (10) 1 (10)
Severe 6 (60) - - - -
None - - - - -
Slight - 1 (10) 5 (50) 7 (70) 9 (90) <0.05
Moderate 5 (50) 6 (60) 5 (50) 3 (30) 1 (10)
Severe 5 (50) 3 (30) - - -
122
effects, so not really an ideal therapy. 10 3. Jossa F, Macinic M. The

There is need and vacuum to find an Mediterranean diet in the prevention
of arteriosclerosis research. Prog Med
alternative drug and the present study is an 1996; 87: 175-81.
attempt to address this topic. 4. Visioli F, Galli C. The effect of minor
constituents of olive oil on
cardiovascular disease: new findings.
Our results show that topical use of olive Nutr Rev 1998; 56: 142-7.
oil is effective in the treatment of lichen 5. Walton AJ, Snaith ML, Ocniskar M et
simplex chronicus. Scanty data are al. Dietary fish oil and the severity of
symptoms in patients with systemic
available on this subject. How does this lupus erythematosus. Ann Rheum Dis
improvement occur? In addition to the 1991; 50: 463-6.
placebo ef fect, other mechanism s may be 6. Linos A, Kaklamanis E,
Kontomerkosa A et al. The effect of
responsible. Olive oil is a good emollient olive oil and fish consumption on
which might help in breaking the itch- rheumatoid arthritis – a case control
scratch-lichenification vicious cycle, the study. Scand J Rheumatol 1991; 20;
419-26.
basic underlying pathophysiology in LSC. 7. Polaano MK, ed. Topical Skin
Different ingredients of olive oil may also Therapeutics. Edinburgh: Churchill
be the other contributory factors. The Livingsstone; 1984.
8. Bjorneboe A, Smith AK, Bjorneboe
major components of olive oil are GE et al. Effect of dietary
monounsaturated oleic acid; squalene, supplementation with n-3 fatty acid
tocopherols.11 These possess anti- on clinical manifestation of psoriasis.
Br J Dermatol 1998; 118 : 77-83.
inflammatory, anti-oxidant and scavenger 9. Pock LW. Essential fatty acid
properties. How far these mechanisms are deficiency in renal failure. Can
helpful in LSC needs to be searched for. supplements really help? J Ann Diet
Assoc 1997; 97: 5150 -3.
Placebo-controlled, double-blind studies 10. Burton JL, Holden CA. Eczema,
are required to determine the comparative lichenification and prurigo. In:
efficacy of olive oil in LSC. Champion RH, Burton JL, Burns DA,
Breathnach SM, eds.
Rook/Wilkinson/Ebling Textbook of
Olive oil was well-tolerated by our dermatology, 6th edn. London:
patients confirming that olive oil has low Blackwell Science; 1998. p. 629-86.
sensitizing potential. 12 11. Composition of olive oil.
www.oliveoil. (accessed on
15.06.04).
In conclusion, olive oil appears to be 12. Kranke B, Komericki P, Aberer W.
effective in the treatment of lichen simplex Olive oil - contact sensitizer or
irritant. Contact Dermatitis 1997; 36:
chronicus; however, further investigations 5-10.
are required to confirm this.
References
1. Khan U, Saeed A, Alam MT, eds.
Olea europaea. Karachi: University
of Karachi; 1997. p. 314 -5.
2. Ghaznavi K, ed. Tibb-e-nabvi Aur
Jadid Science. Lahore: Alfaisal
publishers; 1992.
123
Family history of psoriasis and recent infection…. Shahzana Naqqash et al.
Original article
Family history of psoriasis and recent
infectious disease are risk factors for the
first episode of acute guttate psoriasis
Shahzana Naqqash, Tameez-ud-deen, Shahid Naqqash*, Abdul Quddus Butt
Dermatology Department, Rawalpindi General Hospital, Rawalpindi
*ENT Department, POF Hospital, Wah Cantt
Abstract Background Psoriasis is a heterogeneous disease in its clinical expression. Both genetic
and environmental factors are thought to contribute to the pathogenesis. The association of
guttate psoriasis with streptococcal pharyngitis is well accepted. The association of other
risk factors is less well-defined.
Objectives The aim of this study was to estimate the risk for guttate psoriasis with recent
infections and to explore other potential risk factors like family history of psoriasis.
Patients and methods This was a case-control study. Cases were patients with the first
diagnosis ever of acute guttate psoriasis. Controls were patients newly diagnosed as having
dermatologic conditions other than psoriasis and seen in the same outpatient services as the
cases. Inclusion of cases and controls was restricted to patients up to 18 years of age. A
total 35 cases (median age, 8 years) and 150 controls (median age, 12½ years) were
included in the analysis.
Results A significant difference was observed for a family history of psoriasis. 45.7% of
patients with guttate psoriasis gave a family history of psoriasis in their first-degree
relatives. The risk of psoriasis was also increased in subjects who reported a history of a
recent infectious episode. The analysis by individual diagnosis pointed to acute pharyngitis
as the disease with the strongest association. Twenty-seven patients (77.1%) gave a history
of sore throat preceding the onset of guttate psoriasis. All of them had a throat swab
performed, of these 20 had normal flora cultured. Only 6 had a positive culture and in these
cases Lancefield group C ß-hemolytic streptococci were isolated. ASO titer was raised in
21 (60%) patients of guttate psoriasis .
Conclusion The study confirmed that recent pharyngeal infection is a risk factor for guttate
psoriasis. It also documented the strong association between guttate psoriasis and a family
history of psoriasis
Key words Guttate psoriasis, streptococcal pharyngitis, ASO titer
Introduction comprises immune-mediated cutaneous

inflammation and keratinocyte
Psoriasis is a chronic scaly and
hyperproliferation, and many biochemical
inflammatory skin disorder, the
and immunological abnormalities have
pathogenesis of which remains elusive, but
been identified.1 Psoriasis is a
genetic and environmental factors are
heterogeneous disease and various clinical
thought to contribute. The disease process
subtypes can be differentiated. Guttate
Address for Correspondence psoriasis is characterized by the eruption
Dr. Shahzana Naqqash of small erythematous and scaling lesions
House No. 22, Ravi Road
Wah Cantt, Pakistan. over large areas of the skin surface 1-2
124
weeks after an episode of acute tonsillitis body. Controls were patients who were
or pharyng itis.2 It represents a diagnosed for the first time in their life as
manifestation of psoriasis of an early age having dermatologic conditions other than
at onset and as such is more frequent than psoriasis. They were recruited in the same
other varieties in children and young outpatient services. Inclusion of cases and
adults. It may arise on its own (acute controls was restricted to patients up to 18
guttate psoriasis) or may complicate years of age. A standardized interview was
existing, often quite limited, chronic used to assess the disease onset in cases
plaque psoriasis (guttate flare of chronic and controls. A total 35 cases of guttate
plaque psoriasis). If left untreated, guttate psoriasis, satisfying entry criteria, and 150
psoriasis may clear spontaneously or may controls were recruited.
develop into chronic plaque psoriasis. It
may recur, although the risk is not well- Information about family history of
defined. 3 Acute guttate psoriasis is psoriasis in blood relatives (siblings and
associated with infections by parents), and personal medical history was
Streptococcus pyogenes, and cross- obtained from cases and controls using an
reaction between skin and streptococcal identical structured questionnaire.
antigens have been reported. Information on any infectious disease
requiring at least one medical attendance
The role of superantigens in the during the three months before diagnosis
pathogenesis of psoriasis is a well- was collected in cases and controls.
established and attractive hypothesis.4 Detailed history regarding sore throat was
Super antigens include viral and bacterial recorded, throat swabs were taken in
proteins that can stimulate T -cells to relevant patients and ASO titre was
proliferate without prior intracellular determined in all cases and controls.
processing by an antigen-presenting cell. Anthropometric measures including height
and weight were also obtained.
The aim of our case-control study was to
provide a quantitative estimate of the risk Results
for guttate psoriasis associated a recent
streptococcal throat infection. The patients with guttate psoriasis
consisted of 23 female (65.7%) and 12
Patients and methods male (34.3%) subjects. The control
population consisted of 90 (60%) female
Our case-control study was conducted at and 60 (40%) male subjects. The median
dermatology department, Rawalpindi age at diagnosis was 8 years in cases and
General Hospital, Rawalpindi, from Dec. 12½ years in controls. The distribution of
2002 to Dec. 2003. cases and controls did not show any
substantial differences in the distribution
Entry criteria for cases were the first ever of gender.
diagnosis of acute guttate psoriasis with no
previous diagnosis of psoriasis. Guttate In 20 (57.1%) cases , lesions of guttate
psoriasis was defined as the abrupt psoriasis were combined with classic
appearance of drop-like, round or oval plaque psoriasis. Diagnosis in the control
orange-brown asymptomatic papules group comprised the following: eczema 40
covered with scales and scattered over the (26.7%), urticaria 30 (20%), scabies 50
125
Table 1 Demographic data of study population Discussion

ad controls
Cases Controls This study provides the evidence that

Age (years) guttat e psoriasis, in subjects without a
<5 6 30
5-10 14 70 previous diagnosis of psoriasis is strongly
10-18 15 50 associated with a family history of
Sex (male/female) 12/23 60/90 psoriasis. This is in accordance with
Family history of
psoriasis previous studies. 5, 6 To avoid recall bias in
(parents/siblings) the reporting of a family history of
No 19 130 psoriasis, the study was restricted to newly
Yes 16 20
Recent infectious
diagnosed cases and controls. It is likely
disease that genetic factors are involved. Familial
No 8 125 cases of guttate psoriasis have been
Yes 27 25
described 5,7 and a highly significant
ASO titre
>200 IU ml-1 21 10 association with HLA-Bw17 has been
<200 IU ml-1 14 140 reported in guttate as well as in plaque
psoriasis . 8 Moreover, HLA-B13 has been
(33.3%), superficial mycosis 20 (13.3%) linked to a history of severe streptococcal
and acne 10 (6.7%). The distribution of
infection in these patients. 9 A long held
cases and controls according to age, sex,
belief supported by fairly convincing
family history of first degree relatives clinical immunologic evidence associates
and history of previous and concomitant guttate psoriasis with acute infection,
infection is presented in Table 1. particularly from S. pyogenes. 10,11 Our
study suggests that recent pharyngeal
A significant difference was observed for infection is associated with an increase in
a family history of psoriasis. 16 cases the risk for the first episode of guttate
(45.7%) had a family history of psorias is
compared with subjects not reporting such
whereas only 20 of the control patients a history, as 27 out of 35 patients (77.1%)
(13.3%) had a family history of psoriasis. gave the history sore throat within
The risk of psoriasis was also increased in
preceding three months, the infectious
subjects who reported a history of a recent agent responsible found out to be S.
infectious episode. The analysis by pyogenes. In studies relying on
individual diagnosis pointed to acute bacteriologic culture, S. pyogenes has been
pharyngitis as the disease with the
isolated in a far higher proportion of
strongest association. Twenty-seven
guttate psoriasis patients ranging from
(77.1%) patients gave a history of sore 20% to 97%.12 Group A streptococci are
throat preceding the onset of guttate thought to be responsible for majority of
psoriasis. All of them had a throat swab
cases of streptococcal-induced pharyngitis,
performed, of these 20 had normal flora
but strains of other serogroups, especially
cultured. Only 6 had a positive culture and groups D and G, may occasionally be
in these cases Lancefield group C ß- involved. 13 Confirmation of streptococcal
hemolytic streptococci were isolated. ASO
infection in guttate psoriasis may be
titer was raised in 21 patients (60%), while
difficult because patients are often seen in
it was raised in only 10 of 150 controls the convalescent phase when antibiotics
(6.66%). The clinical features of cases have already been prescribed 2 and throat
studied are given in Table 2.
swab cultures are more likely to be
126
Table 2 Clinical features of guttate psoriasis
Subject Age Sex Family History of sore Throat swab ASO titre
(years) History throat culture (IU ml -
1b
)
c
1 1 m No Yes n/d <200
2 7 f Yes Yes normale 800
3 9 m Yes Yes normal 400
4 9 f No Yes normal 300
6 2 f No No normal <200
7 14 f Yes Yes Gp C 400
8 7 m No No n/d 250
9 17 f Yes Yes normal 300
11 18 f Yes Yes normal <200
13 10 m Yes Yes Gp C 1200
16 5 m Yes Yes normal <200
17 3 f Yes No n/d <200
18 7 f Yes Yes n/d <200
19 13 m Yes Yes Gp Cg 1600
21 6 f No Yes n/d <200
22 8 m No Yes normal 1500
23 11 m No No n/d <200
25 3 f Yes Yes Gp C 800
26 7 f Yes No normal 400
27 5 f Yes Yes normal <200
28 14 m No Yes normal <200
30 17 m No Yes Gp C 800
31 10 f Yes No normal 400
32 6 f No Yes Gp C 1200
33 7 m No No n/d <200
35 3 m No No n/d <200
a
Preceding onset pf psoriasis. b Normal antistreptolysin O titre is <200 IU ml-1 . c n/d = not done. d GP =
guttate psoriasis. e Normal = normal flora isolated. f CPP = chronic plaque psoriasis. g Lancefield group
C ß-hemolytic streptococcus isolated. h Anti-DNase B
negative. Approximately 20% of group A throat culture. There are limited data
streptococcal infected individuals do not available on the results of investigation
respond by so that a negative titer alone into the association between streptococcal
streptococcal infection. 14 infection and guttate psoriasis; serological
evidence of streptococcal infection was
Sixty per cent of subjects in this study had found in 19 of 33 (58%) with acute guttate
raised ASO titers at presentation while psoriasis in one study.2 In another study
only six of 27 patients investigated with a Mallon et al.15 27twenty seven of 29
throat swab had a positive streptococcal patients (93%) had raised ASO titer . In our
127
study, 60% of patients with guttate management of acute guttate psoriasis.

psoriasis had raised ASO titer and 17.1% Furthermore, it is not clear whether any
had positive culture of S pyogenes. It is intervention can effectively, prolong the
unlikely that these are mere chance duration of remission or prevent
associations. There is evidence that an progression to chronic plaque psoriasis.
immunologic mechanism is involved in More studies are needed with greater
the triggering of guttate psoriasis by numbers of patients so that risk
streptococcal infection. Stimulation of T- associations of psoriasis can be determined
cells by streptococcal superantigens has accurately, and optimal method for
been suggested. 11,16,17, 18 They bind directly achieving clearance of psoriasis can be
to the major histocompatibility complex determined.
class II molecule on the antigen presenting
cell and stimulate T cells that express References
1. Van de Kerkhof PCM. Boss JP, eds.
certain T-cell receptors. This leads to Pathogenesis aspects of psoriasis.
polyclonal T-cell activation with release of Clin Dermatol 1995; 13: 97-8.
immune cytokines such as interleukin-2, 2. Telfer NR, Chalmers RJ, Whale K,
Colman G. The role of streptococcal
which are important in the pathogenesis of infection in the initiation of guttate
psoriasis. 19 It is possible that streptococci psoriasis. Arch Dermatol 1992; 128:
contain antigenic substances recognized 39-42.
3. Martin BA, Chalmers RJC, Telfer
by psoriatic T-cells. Aiba et al. 20 and NR. How great is the risk of further
Baker et al.21 reported the altered psoriasis following a single episode of
responses of peripheral blood acute guttate psoriasis? Arch
Dermatol 1996; 132: 717 -8.
mononuclear cells (PBLs) from psoriatic
4. Valdimarsson H, Baker BS, Jonsdottir
patients to streptococcal antigen in vitro. I et al. Psoriasis: a T-cell mediated
Furthermore, Baker et al.22 have reported autoimmune disease induced by
the presence of streptococcal antigen- streptococcal superantigens? Immunol
Today 1995; 16: 145-9.
specific T-cells in guttate psoriasis lesions. 5. Banno T, Fujisawa H, Stomi H et al.
Gabriel et al. 23 confirmed the auto-immune Psoriasis vulgaris and acute guttate
components in guttate psoriasis, due to psoriasis in a family. Int J Dermatol
2001; 40: 285-7.
cross reactions between skin and 6. Luigi N, Lorenzo P, Fabio P, Claude
streptococcal antigens . FC, and the psoriasis study group of
the Italian Group for Epidemiological
Research in Dermatology. Family
Keeping these studies in mind, and the history of psoriasis, stressful life
strong association documented in our events, and recent infectious disease
study, between guttate psoriasis and recent are risk factors for a first episode of
acute guttate psoriasis: Results of a
acute pharyngitis, it is important to search case-control study. J Am Acad
for and eliminate microbial infections in Dermatol 2001; 44: 433-8.
the treatment of psoriasis. In view of this 7. Bolton GG, Daniel CR. A family
outbreak of acute guttate psoriasis .
many dermatologists have recommended Arch Dermatol 1990; 126 : 1523-4.
using antibiotics for psoriasis particularly 8. Williams RC, Mckenzie AW, Roger
guttate type. Some dermatologists have JH, Joysey VC. HLA -A antigens in
patients with guttate psoriasis. Br J
also recommended tonsillectomy for Dermatol 1976; 95: 163-7.
psoriasis in patients with recurrent 9. Krain LS, Newcomer VD, Terasaki
streptococcal pharyngitis. There is, PI. HLA antigen in psoriasis [letter].
N Engl J Med 1973; 288: 1245.
currently, no firm evidence on which to
base any recommendations for the routine
128
10. Wilson AG, Clark I, Heard SR et al. antigens in chronic plaque psoriasis.
Immuno-blotting of streptococcal Br J Dermatol 1991; 125 : 38-42.
antigens in guttate psoriasis. Br J 22. Baker BS, Powles A, Garioch JJ et al.
Dermatol 1993; 128: 151-8. Group A streptococcal antigen-
11. Leung DY, Travers JB Giorno R et al. specific T lymphocytes in guttate
Evidence for a streptococcal psoriatic lesions. Br J Dermatol 1993;
superantigen-driven process in acute 128 : 493-9.
guttate psoriasis. J Clin Invest 1995; 23. Villeda GG, Santamaria CLC, Perez
96: 2106-12. LR et al. Recognition of
12. Tervaert WC, Esseveld H. A study of Streptococcus pyogenes and skin
the incidence of haemolytic autoantigens in guttate psoriasis. Arch
streptococci in the throat in patients Med Res 1998; 29: 143-8.
with psoriasis vulgaris with reference
to their role in the pathogenesis of this
disease. Dermatologica 1970; 140:
282-90.
13. Stjernquist-Desatnik A, Prellner K,
Christensen P. Clinical and laboratory
findings in patients with acute
tonsillitis. Acta Otolaryngol (Stockh)
1987; 104 : 351-9.
14. Johnson DR, Kaplan EL, Stramek J et
al., eds. Laboratory diagnosis of
group A streptococcal infections.
Geneva: World Health Organization;
1996.
15. Mallon E, Bunce M, Savoie H et al.
HLA-C and guttate psoriasis. Br J
Dermatol 2000; 143: 1177-82.
16. Leung DY, Walsh P, Giorno R,
Norris DA. A potential role for
superantigens in the pathogenesis of
psoriasis. J Invest Dermatol 1993;
100 : 225-8.
17. Lewis HM, Baker BS, Bokth S et al.
Restricted T cell receptor V-beta gene
usage in the skin of patients with
guttate and chronic plaque psoriasis.
Br J Dermatol 1993; 129 : 514 -20.
18. Horiuchi N, Aiba S, Ozawa H et al.
Peripheral blood lymphocytes from
psoriatic patients are hyporesponsive
to beta-streptococcal superantigens.
Br J Dermatol 1998; 138 : 229 -35.
19. Skov L, Baadsgaard O.
Superantigens. Do they have a role in
skin diseases? Arch Dermatol 1995;
131 : 829-32.
20. Aiba S, Tagami H. Proliferative
responses of peripheral blood
mononuclear cells from psoriatic
patients to T lymphocyte-stimulating
cytokines (IL -2, IL-3, IL-4 and
granulocyte-macrophage colony-
stimulating factor) and OK-432. Arch
Dermatol Res 1989; 281 : 310-5
21. Baker BS, Powel AV, Malkani AK et
al. Altered cell-mediated immunity to
group A haemolytic streptococcal
129
Porokeratosis: a review….. Arfan ul Bari and Simeen Ber Rahman
Re view article
Porokeratosis: a review of unique group of
keratinizing disorder
Arfan ul Bari, Simeen Ber Rahman*
Consultant Dermatologist, PAF Hospital, Sargodha
* Dermatology Department, Military Hospital, Rawalpindi
Abstract Porokeratosis is a group of disorder of uncertain cause characterized by abnormal epidermal

keratinization with the histologic finding of cornoid lamella. To date, five major clinical variants
have been identified. This unique group of disorders of keratinization is reviewed here, with special
reference to the differentiation of each component and their management.
Key words
Porokeratosis, porokeratosis of Mibelli, disseminated superficial actinic porokeratosis, porokeratosis
palmaris et plantaris disseminate, linear porokeratosis, punctate porokeratosis, cornoid lamella.
Introduction seen at any age, from birth to adulthood, PM

Porokeratosis is a clonal disorder of usually develops in childhood, DSAP generally
keratinization characterized by one or more develops in the third or fourth decade of life.1,2
atrophic patches surrounded by a clinically and Lesions may be found anywhere, including the
histologically distinctive ridge-like border called mucous membranes, although they most
the cornoid lamella. Five clinical variants of commonly occur on the extremities.3-5 A
porokeratosis are recognized: (i) classic verrucous variant that is localized to the buttocks
porokeratosis of Mibelli (PM), (ii) disseminated and resembles psoriasis has been reported in
superficial actinic porokeratosis (DSAP), (iii) several patients6. Several risk factors for the
porokeratosis palmaris et plantaris disseminate development of porokeratosis have been
(PPPD), (iv) linear porokeratosis (LP), and (v) identified; these include genetic inheritance,
punctate porokeratosis (PP). Porokeratosis most ultraviolet radiation, and immunosuppression.
commonly occurs in fair-skinned individuals and Excessive natural or artificial ultraviolet
is relatively rare in darker-skinned races. PM radiation, electron beam therapy, and extensive
and PPPD affect men twice as often as women. radiation therapy are well-established trigger
DSAP is three times more common in women factors. Immunosuppression may induce new
compared with men and LP is seen with equal lesions or cause preexisting lesions to flare.2,7-10
incidence in both sexes. PPPD and LP may be The approach to treatment is individualized,
based on the size of the lesion and the
Address for Correspondence anatomical location, the functional and aesthetic
Squadron Leader Dr. Arfan ul Bari,
Consultant Dermatologist, PAF Hospital, considerations, the risk of malignancy, and the
Sargodha patient’s preference. Protection from the sun,
Ph# (off): 0451-5553307, (res): 0451-5553308
E mail: albariul@yahoo.com use of emollients, and watchful observation for
130
signs of malignant degeneration may be all that dominant mode of inheritance has been
is needed for many patients. Various medical reasonably well established for PM,27,28 PPPD,26
and surgical modalities are also available. DSP, and DSAP.29 Linear porokeratosis has been
Excision is most appropriate when malignant observed in monozygotic twins.30 The
degeneration develops. Cryotherapy, similarities of clinical appearance and
electrodesiccation and curettage are minimally histopathology as well as the coexistence of
invasive methods of inducing resolution for different variants of porokeratosis in one patient
large numbers of lesions. Diamond fraise or in several members of an affected family
dermabrasion and laser therapy has also been make a strong case for considering them
used with conflicting reports of efficacy.11-16 The different phenotypic expressions of a common
prognosis is generally excellent.2 Patients who genetic aberration.31,32 Risk factors for
develop PM or linear porokeratosis because of porokeratosis include genetic inheritance,
immunosuppression are at higher risk for the ultraviolet light exposure, and
development of a squamous or basal cell immunosuppression. One study found that
carcinoma within the lesion. Linear approximately 10% of patients who had
porokeratosis is associated with a higher risk of undergone renal transplantation developed
malignant degeneration.17-19 PM porokeratosis.1,7-10 An autosomal dominant mode
circumferentially involving the digits may of inheritance has been established for familial
induce pseudoainhum.20 Patients must practice cases of almost all forms.
strict sun precautions and must periodically
examine their skin for lesions suggestive of Classic porokeratosis (Mibelli) Autosomal
malignancy.
dominant inheritance and immunosuppression
are the usual causes.27,28 PM has also been seen
Historical Background following radiation therapy, at burn wounds, and
at hemodialysis sites.33
Porokeratosis was first described by Mibelli21 in

1893 as one or more localized, chronically Disseminated superficial (actinic)
progressive, hypertrophic irregular plaques with porokeratosis Sun exposure and/or artificial
central atrophy and a prominent peripheral ridge. ultraviolet radiation exposure in a patient who is
A more superficial disseminated form was genetically predisposed cause DSAP.
described independently almost at the same time Exacerbations have been reported following
by Respighi22 and later by Andrews.23 A linear prolonged sun exposure, repeated tanning bed
variant was added early in the last century.24 exposure, electron beam radiation therapy, and
Disseminated superficial actinic porokeratosis 25 therapeutic phototherapy or photochemotherapy
was described in 1966 and porokeratosis for psoriasis.34,35 Drug-induced photosensitivity
palmaris et plantaris disseminate26 was added to may play a role. Protection from ultraviolet
the spectrum in 1971. radiation may lead to spontaneous resolution.
Immunosuppression predisposes patients to both
DSAP and nonactinic DSP.36 Because of this, a
Etiology and Pathogenesis viral etiology has been hypothesized.
The exact etiology of the various types of
porokeratosis is unknown. An autosomal
131
Linear porokeratosis No definite inheritance anywhere, including the mucous membranes,

pattern has been established. Loss of although they most commonly occur on the
heterozygosity has been proposed as a genetic extremities.1,3,4-6,27,28,33
mechanism and may explain the higher risk of
malignant degeneration seen in linear 2. Disseminated superficial (actinic)
porokeratosis in comparison to other forms of porokeratosis [Figure 2]
porokeratosis.30,37
Multiple, brown, annular, keratotic lesions that
develop predominantly on the extensor surfaces
Porokeratosis palmaris et plantaris disseminate of the legs and the arms characterize DSAP.
Familial PPPD is transmitted in an autosomal They are usually asymptomatic, but they may
dominant mode with variable penetrance.26 itch slightly. Facial lesions are seen in
Acquired PPPD may be caused by approximately 15% of patients, and the face may
immunosuppression, or it may be a cutaneous be the only area of involvement. Patients are
marker of internal malignancy.38
typically women in their third or fourth decade
of life, with a history of excessive ultraviolet
Punctate porokeratosis This condition has no exposure. Patients may have a history of
unique inheritance pattern and is usually phototherapy for psoriasis.1,8,19,31,34,35
associated with other forms of porokeratosis.39
3. Non-actinic disseminated superficial
Clinical Variants porokeratosis
1. Porokeratosis of Mibelli [Figure 1] Non-actinic forms may be seen following

electron beam total skin irradiation, organ
It generally starts in childhood as a small, transplantation, hepatitis C virus related
asymptomatic or slightly pruritic lesion that hepatocellular carcinoma, HIV infection, renal
expand over a period of years, but may develop failure, or in association with other causes of
during adulthood and enlarge rapidly, usually in immunosuppression. Dozens of small, indistinct,
the clinical setting of immunosuppression. light brown patches with a threadlike border are
Occasionally, patients have a history of an seen on the extensor surfaces of the arms and the
antecedent trauma, such as a burn wound. The legs. Non actinic DSP may have a generalized
lesion develops as a small, light brown, keratotic distribution, sparing the palms and the soles.34,40-
papule that slowly expands to form an 42
irregularly shaped, annular plaque with a raised,

4. Linear porokeratosis [Figure 3]
ridgelike border. This border may be
hypertrophic or verrucous and is usually greater During infancy or early childhood, a unilateral,
than 1 mm in height. A thin furrow is typically linear array of papules and plaques with the
seen in the center of the ridge, causing a Great characteristic raised peripheral ridge are seen
Wall of China effect. The lesion is slightly unilaterally on an extremity, the trunk, and/or
hypopigmented or hyperpigmented, minimally the head and neck area. The lesions commonly
scaly, slightly atrophic, hairless, and anhidrotic. follow a dermatomal distribution. Multiple
The size may vary from a few millimeters to linear groups may be seen in one patient,
several centimeters. Lesions may be found typically on the same side. They may be seen in
132
Figure 4 Porokeratosis palmaris plantaris et

Figure 1 Porokeratosis of Mibelli over central face of disseminate lesions over planter region.
an elderly person.
Figure 2 Disseminated superficial actinic

porokeratosis over forearm.
Figure 5 Porokeratosis palmaris plantaris et

disseminate lesions over calf region.
association with other forms of porokeratosis.

Individual lesions within the linear grouping
have a well-developed border, often with a
central furrow similar to that seen in classic PM.
Clinical changes consistent with the
development of a basal or squamous cell
carcinoma are more common in linear
porokeratosis than in other forms of
Figure 3 Linear porokeratosis over hand. porokeratosis.1,17,24,30,37
133
5. Porokeratosis palmaris et plantaris have a high incidence of malignant

disseminate [Figures 4, 5] transformation.32,49
Small, relatively uniform lesions are first seen
on the palms and the soles, and then they 9. Bullous porokeratosis
develop in a generalized distribution, including This has been described in association with
the mucosal membranes. The lesions may itch or disseminated superficial porokeratosis .50
sting, but they are usually asymptomatic. The
onset is typically during adolescence or early
10. Pruriginous porokeratosis
adulthood, and males are affected twice as often
as females. The lesions are small and superficial This variant has again been described in association
with disseminated superficial porokeratosis .50,51
with a slightly hyperpigmented, atrophic center
and a minimally raised peripheral ridge. 11. Zosteriform porokeratosis
Mucosal lesions are small, annular or Porokeratotic lesions have rarely been seen in
serpiginous, and pale. Squamous cell carcinoma dermatomal pattern.52
has been reported to develop within lesions of
PPPD.1,26,38,43,44 12. Mutilating variant
Mutilating and destructive lesions have been
6. Punctate porokeratosis reported in PM.53,54
Multiple, asymptomatic, tiny, seed-like, 13. Malignant porokeratosis

hyperkeratotic papules with thin, raised margins Any porokeratosis developing fatal malignancies
develop on the palms and the soles during may be regarded as malignant porokeratosis.55
adulthood. Patients usually have other forms of
porokeratosis as well, most commonly the linear Association with cutaneous malignancies
or Mibelli types. Punctate porokeratosis may be
The exact molecular mechanism of
clinically and histologically indistinguishable
carcinogenesis developing in various forms of
from punctate porokeratotic keratoderma, which
porokeratosis remains unclear, but chromosomal
may be a cutaneous sign of an internal
instability and reduced immune surveillance
malignancy.39,45,46
with over expression of p53 are hypothesized to
play a role in the development of cutaneous
7. Verrucous /hyperkeratotic variant malignancies within porokeratosis. Sudden
A verrucous variant that is localized to the aggravation of DSP and DSAP should prompt a
buttocks and resembles psoriasis has been search for an underlying disease causing
reported in several patients. Several cases of immunosuppression. Squamous cell carcinoma,
hyperkeratotic variants of PM and DSAP have Bowen's disease and basal cell epithelioma have
also bee described.47,48 been observed and are more likely in large
isolated lesions of PM, but malignant
transsformation has also been observed in DSP
8. Giant porokeratosis
and DSAP and linear porokeratosis. Widespread
Rarely the lesions of porokeratosis may be 10-20 metastases and fatal outcome have rarely been
cm in diameter and the surrounding wall raised 1 reported.1,2,7,19,56-60
cm. Mostly found on the foot and are said to
134
Histopathology [Figure 6, 7]
Porokeratoses are grouped because of their
histologic hallmark, the cornoid lamella, and the
resulting clinical features. It has been proposed
that, in porokeratosis, a mutant clone of
epidermal cells expands peripherally, leading to
formation of a cornoid lamella at the boundary
between the clonal population and normal
keratinocytes and represents the pathologic
Figure 6 Characteristic histology showing two
substrate for the ridge-like border. The cornoid parallel column of compact
lamella arises in the interfollicular epidermis and parakeratotic/dyskeratotic cells (cornoid lamellae)
may involve the ostia of hair follicles or sweat indenting the underlying epidermis.
ducts, which have led to the misnomer
porokeratosis. It consists of a tightly packed,
thin column of parakeratotic cells extending
through the entire thickness of the surrounding
orthokeratotic stratum corneum. It occupies an
indentation of the epidermis that is generally
tilted away from the center of the lesion. The
adjacent epidermis is hyperkeratotic and
acanthotic to a variable degree. The granular
layer is missing below the cornoid lamella, and
single or clustered dyskeratotic cells and Figure 9 High power view of (cornoid lamellae) with
vacuolated keratinocytes are found at its base. absence of granular layer and liquefaction in basal
The papillary dermis beneath the cornoid layer.
lamella contains a moderately dense
inflammatory infiltrate and dilated capillaries. continuous keratotic ridge cleaved by a
The center of the lesion is usually atrophic, with longitudinal furrow, which surrounds the lesions
areas of liquefaction degeneration in the basal both in the Mibelli type and the other variants, is
layer, colloid body formation, and flattening of quite diagnostic, as is the localization and
rete ridges, whereas the dermis may be distribution of lesions. Elastosis perforans
edematous or fibrotic with telangiectasia. In serpiginosa can be similar to PM, but it consists
essence, similar histopathologic changes are of erythematous, keratotic papules and lacks the
encountered in all forms of porokeratosis. continuous ridge with its furrow. The
However, in DSP, DSAP and PPPD, the cornoid superficial, disseminated types of porokeratosis
lamella is less pronounced or so minimal as to may resemble actinic keratoses, stucco
be difficult to recognize.1,21,60,61 keratoses, flat seborrheic keratoses, or flat warts.
Small, discrete lesions may be mistaken for
lichen sclerosus et atrophicus, lichen planus,
Diagnosis and differential diagnosis
acrokeratosis verruciformis, and pityriasis rubra
Diagnosis of porokeratosis is usually made with pilaris, but each of these others lacks the fine,
ease, both clinically and histopathologically. The slightly raised, threadlike border. Neoplastic
135
disorders, such as cutaneous T cell lymphoma Course and Prognosis

(CTCL), can mimic lesions DSAP or PM Prognosis is generally excellent in patients
clinically. Histologically, the lesions lack a having no underlying immunosuppression and
cornoid lamella and reveal infiltrates typical of paucity of risk factors. Lesions may increase in
CTCL. Punctate porokeratosis should be size and number with time; while this may be an
differentiated from punctate keratoderma and extremely slow process in PM, progression can
planter warts.1,6,62,63
be quite pronounced in DSP and particularly in
DSAP after UV exposure. In
Treatment immunocompromised patients, spontaneous
fluctuations in severity and spontaneous
The optimal treatment modality must be selected remissions depending on the immune status have
depending on the lesion's size and localization, been described. Sudden aggravation of DSP and
functional and aesthetic requirements, and the DSAP should prompt a search for an underlying
general condition of the patient. The lesion can disease causing immunosuppression. Malignant
recur with any therapeutic modality. Lubrication degeneration has been observed and is more
usually improves the symptoms in superficial likely in large isolated lesions of PM, but it has
forms of porokeratosis, as does keratolytic also been observed in DSP and DSAP and linear
treatment of hyperkeratotic lesions. Topical 5- porokeratosis. Widespread metastases and fatal
fluorouracil is highly effective in PM, linear outcome have been reported. Several cases of
porokeratosis, and in DSP and DSAP. Treatment giant PM in an acral location causing destruction
must be continued until a brisk inflammatory of underlying soft tissue and bone and mutilation
reaction is obtained that seems to be a have been observed.17-20,53-55,64-66
prerequisite for clearing and can occur as a
delayed reaction. The use of oral retinoids has
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Editor’s note
From the next issue of JPAD, the ‘QUIZ’ section will be replaced by
‘PHOTODERMDIAGNOSIS’ section and it will be edited by Dr. Amor
Khachemoune, MD. The manuscripts should be submitted to:
Dr. Amor Khachemoune, MD. 1440 Beacon St # 508, Brookline MA 02445,
USA.
E-mail: amork@pol.net.
139
Management of atopic dermatitis…. Amer Ejaz and Naeem Raza.
Review article
Management of atopic dermatitis – A
review
Amer Ejaz, Naeem Raza
Consultant Dermatologist, Combined Military Hospital, Malir, Karachi
* Consultant Dermatologist, Combined Military Hospital, Abbottabad
Abstract Atopic dermatitis is a chronic relapsing and remitting disease, which afflicts mostly the
young ones. The treatment of atopic dermatitis is very frustrating as no single
therapeutic modality is satisfactory. The first breakthrough in its management came
with the advent of topical steroids. We are still awaiting the next breakthrough. Much
research is being done in this regard and the pick of the lot is topical immunotherapy.
It may well prove to be the find of the decade in the management of atopic dermatitis.
This article summarizes the established as well as unconventional therapeutic
modalities which help the patients as well as which has evidence of successful use.
Key words
Atopic dermatitis, management
Introduction to educate the patient and family in certain

realities of their disease.
Atopic dermatitis is a chronic relapsing 1. The disease runs its own course and
and remitting disorder, which usually physicians can only moderate that course;
starts in early childhood. It is frequently the disorder is not curable but it is always
associated with elevated levels of serum possible to improve the patients' condition.
IgE and a personal or family history of 2. The skin must be kept moisturized.
atopy i.e. allergic rhinitis, atopic dermatitis 3. It is crucial that trigger factors are
or asthma. The diagnosis is clinical and avoided in order to prevent inflammation.
based on a combination of historic and
morphologic finding, as there is no single a. Mild cleansing
distinguishing feature. 1 Atopic dermatitis Routine everyday care of skin is an
now affects 15% to 20% of children in essential part of optimal patient
developed countries, and prevalence in management in atopic dermatitis. Xerosis,
cities in developing countries undergoing which is an integral part of atopic
rapid demographic changes is quickly dermatitis, leaves the skin vulnerable to
following suit. 2 The condition also creates external insults, partly as a result of
a great financial burden for both the family varying levels of barrier dysfunction.
and society. 3 Cleansing removes dead surface cells,
preparing skin to better absorb topically
General measures applied drugs/medication. Cleansers based
on mild synthetic surfactants and/or
Perhaps the most important consideration emollients are ideal for these patients.4
in the management of atopic dermatitis is
b. Emollients
Dr. Amer Ejaz, Emollients and moisturizing creams are
Consultant Dermatologist, used to break the dry skin cycle and to
Combined Military Hospital, Malir, Karachi
Email: amer_ejaz@yahoo.com maintain the smoothness of the skin. In the
140
vast majority of cases, improved bathing inhibition of leucocyte recruitment and

techniques and proper moisturizing can activation, and by the reduction of ICAM-
solve the problem. Patients need to avoid 1 expression on keratinocytes. 9,10
hot water, overuse of soap and
unnecessary soaking and rubbing with b. Topical steroids
washcloth and towel. Excessive frequency Topical corticosteroids have been the
of bathing and hand washing should be mainstay of treatment for atopic dermatitis
discouraged. Most importantly, the skin over the past 40 years. Hydrocortisone was
hydration obtained from bathing should be the first to be used; some 30 additional
retained by prompt application of an corticosteroid compounds have now been
adequate moisturizer. The least expensive licensed for treatment of atopic dermatitis.
moisturizers are mineral oil and Topical applications containing
petrolatum etc. Moisturizers should be corticosteroid compounds vary greatly in
applied lightly after the bath and any potency. In general the more potent ones
excess removed with a cotton towel. are associated with the greater risk of
Creams and lotions may be irritating and adverse effects. First application of potent
drying due to an evaporative effect. topical steroids results in rapid clearance
Greasy agents should be avoided in hot of the rash. The snag is that persistent
humid climate to prevent occlusive application of a potent preparation will put
folliculitis. Similarly plastic wrap the patient at risk of unwanted local effects
occlusion may also cause folliculitis.5 on the skin. The development of side
Recently it has been shown that ceramide effects is directly proportional to the
deficiency is the putative cause of stratum frequency of application of topical
corneum dysfunction. Ceramide-dominant, steroids. However, recent trials suggest
barrier repair emollient has been shown to that only once daily application can give
be better than simple emollients. 6 optimal results, 11 and maintenance
treatment can be done effectively with
Established therapies only twice weekly application thus
minimizing the side effects. 12
a. Antihistamines
Oral antihistamines provide only marginal c. UVA therapy
antipruritic benefit and much of the effect Several studies have demonstrated the
seems to be as a sleep aid. Doxepin is the efficacy of UVA1 (340-400 nm)
most potent histamine antagonist and is phototherapy for patients with severe
very specific. So is hydroxyzine, which is atopic dermatitis. However, the optimum
one of the few shown to be effective in a treatment dose has yet to be determined.
controlled trial. However, relief of pruritus Although in seminal investigations high
by non-sedating antihistamines has been UVA1 doses were used, comparable
refuted. 7 Several studies demonstrate the results were reported in recent studies with
efficacy of second and third generation a medium-dose regimen. 13 However,
anti histamines in relieving pruritus in effectiveness is merely short term, limited,
atopic dermatitis . 7,8 but double-blind and is followed by recurrence of
placebo-controlled trial are lacking. 6 symptoms within a 3-month observation
Cetrizine, a second generation interval. 14
antihistamine, has been shown to possess
anti-inflammatory properties, acting via
141
d. UVB therapy inflammatory dermatoses. Tacrolimus

Recently, interest has been renewed in the (FK506), as well as the newer ascomycin
narrow -band wavelength of UVB derivative ASM 981 (pimecrolimus),
phototherapy for atopic dermatitis (TL-01 penetrate the inflamed epidermis and are
Lamp). Encouraging results have been suitable for topical therapy. Both
reported. However, long-term follow-ups substances inhibit the transcription of
are required before this therapeutic proinflammatory cytokin e genes such as
modality is established as treatment of interleukin 2, which are dependent on the
atopic dermatitis . 15,16 nuclear factor NF-AT. They block the
catalytic function of calcineurin, which
e. Antibacterials leads to the inhibition of the transport of
Secondary bacterial infections are known the cytoplasmic component of NF-AT to
to complicate atopic dermatitis, due to the cell nucleus. Multicenter, randomized,
deranged skin barrier function and double blind clinical trials with topical
impaired immunological status. formulations have shown the efficacy of
Staphylococcus aureus is the infecting both substances in moderate to severe
organism in over 99% of episodes. It is atopic dermatitis. 21,22
nearly always penicillin-resistant.
Cloxacillin and erythromycin are c. Montelukast
reasonable choices of treatment. Recent Cysteinyl leukotrienes have been shown to
research has greatly contributed to our be important in the pathogenesis of
understanding of the pathophysiological allergen-induced (atopic) asthma and
potential of S. aureus superantigens in rhinitis. Skin manifestations of atopic
atopic dermatitis, suggesting that dermatitis have been reported to improve
antibiotic therapy might be an important with leukotriene antagonists. Double-blind
element in the therapeutic management of placebo-controlled trials have shown
atopic dermatitis.17 moderate response to montelucast in
severe atopic dermatitis.23,24,25
Newer modalities
d. Photopheresis
a. Cyclosporin Recently, photopheresis was used as
Oral cyclosporin is now an established monotherapy in patients with intractable
therapeutic modality in severe and atopic dermatitis. A total of ten treatments
recalcitrant atopic dermatitis. Several were used with a two weekly interval. All
studies have shown that short-term patients showed clinical improvement as
treatment is effective and tolerable as the well as reduction in serum levels of
drug is tapered off before side effects set eosinophil cationic proteins and total IgE.
26
in. Dosage regimen of 3-5 mg/kg/day for
10 to 12 weeks is effective. 18,19,20
Experimental modalities
b. Topical macrolactum
immunomodulators a. Antifungals
The immunomodulatory macrolactams As fungal colonization is increased in
provide an alternative to atopic dermatitis, there is a rationale to use
glucocorticosteroids for the topical antifungals. Clinical improvement and
treatment of atopic dermatitis and other decreased serum IgE were obtained in
patients with positive Malassezia
142
radioallergosorbent tests (RASTs), who e. Naphthalan

were treated with oral ketoconazole. Some Naphthalan has long been known for its
preliminary data suggested that oral medicinal properties and beneficial effects
itraconazole treatment in AD patients in inflammatory diseases such as psoriasis,
reduced the need for topical atopic dermatitis, and psoriatic dermatitis.
corticosteroids. Furthermore, besides its Experience acquired to date in the use of
antifungal action, itraconazole in part naphthalan in the management of
relieves pruritus and inflammation. 27 squamous dermatoses and atopic
dermatitis has shown favorable results. It
b. Balneotherapy should be further investigated to confirm
Balneotherapy involves immersion of the its usage in the treatment of these
patient in mineral water baths or pools. diseases. 31
Bathing in water with a high salt
concentration is safe, effective, and f. Probiotics
pleasant for healing and recovery. There Recent studies suggest that oral
are almost no side effects during and after bacteriotherapy with probiotics might be
treatment, and there is a very low risk to useful in the management of atopic
the patient's general health and well-being. dermatitis. Lactobacillus GG (ATCC
The mechanisms by which broad 53103), which is a human intestinal strain,
spectrums of diseases are alleviated by spa promotes local antigen-specific immune
therapy have not been fully elucidated. 28 responses (particularly in the IgA class),
Such therapies are being used in many prevents permeability defects, and confers
parts of Pakistan since ancient times. controlled antigen absorption, when given
Many places are known for their hot to infants with atopic eczema and cow's
springs and people travel from great milk allergy as an extensively hydrolyzed
distances to bathe in these waters. whey formula containing (5 × 108 colony-
forming units/gm formula). Thereby it
c. Dinitrochlorobenzene promotes endogenous barrier mechanisms
A small uncontrolled pilot trial has in patients with atopic dermatitis and food
suggested that contact sensitization to allergy by alleviating intestinal
dinitrochlorobenzene and repeated weekly inflammation. 32-34
applications significantly improve the

clinical status of severe atopic dermatitis g. Interferon gamma
in adults. The effects are thought to be due Recombinant human interferon gamma
to topical immune modulation by has been shown in a double-blind,
dinitrochlorobenzene. Larger controlled placebo-controlled study to be a well-
studies of dinitrochlorobenzene treatment tolerated and effective agent in the long-
in atopic dermatitis are warranted. 29 term therapy of patients with AD, when
given in a dose of 50 µg/m2 as daily self-
d. Intradermal Mycobacterium administered subcutaneous injection for up
suspension to 24 months. 35
Recently a small randomized, double blind
and placebo controlled trial has shown h. Mycophenolate mofetil
improvement in childhood atopic Mycophenolate mofetil is a new
dermatitis using intradermal injection of immunosuppressive drug that is used to
killed Mycobacterium vaccae (SRL 172).30 prevent acute rejection of renal
143
transplants. In doses of 2 or 3 g/d the drug special silk fabric (MICROAIR

is well tolerated, effective and safe in non Dermasilk) has been developed to
responding atopic dermatitis.36 minimize such irritant reactions. It has
been shown that wearing such fabric
Comple mentary and alternative minimizes symptoms in atopic children. 42
medicine
e. St. John's wort cream
a. Acupuncture Recent investigations suggest an anti-
Acupuncture is an old therapeutic method inflammatory and antibacterial effect of
that includes both needle and nonneedle hyperforin, which is a major constituent of
acupuncture. Nonneedle acupuncture Hypericum perforatum L. (Saint John's
includes moxibustion, cupping, and wort). A double-blind, placebo-controlled
acupressure. Acupuncture has been prospective study has shown a significant
reported to be beneficial for the treatment superiority of the hyperforin 1.5% cream
of atopic dermatitis among many other compared to the vehicle in the topical
skin conditions. A lack of controlled treatment of mild to moderate atopic
studies is the main drawback for this dermatitis. The therapeutic efficacy of the
method of treatment. However, the hypericum-cream, however, has to be
experiences from experts in this field may evaluated in further studies with larger
offer us new ideas to resolve refractory patient cohorts, in comparison to
disorders in dermatology. 37 therapeutic standards i.e. glucocorticoids. 43
b. Traditional Chinese medicine f. Hypnosis

Traditional Chinese medicine (TCM) is an Hypnosis is an alternative or
alternative method of therapy that can be complementary therapy that has been used
administered in oral, topical, or injectable since ancient times to treat medical and
forms. It emphasizes the importance of dermatologic problems. A wide spectrum
using many herbs that are combined in of dermatologic disorders may be
different formulations for each individual improved or cured using hypnosis as an
patient. In the future, perhaps a better alternative or complementary therapy,
understanding of TCM will be gained including atopic dermatitis. 44
through more systematic analysis and
controlled studies with a placebo arm. 38,39 g. Oolong tea
Standard treatment fails in many patients
c. Homeopathic treatment with recalcitrant AD skin lesions. Study
Homeopathy is one of the commonest results in animal models have
alternative methods of treatment being demonstrated that the administrat ion of tea
used these days by dermatologic patients. (i.e. green, black, or oolong 46) suppressed
Conflicting results of this treatment type I and type IV allergic reactions. This
modality have been reported. Randomized, hypothesis has been tested clinically in an
blinded and placebo controlled trials are open study and oolong tea has been found
lacking in literature. Maybe a few good to be effective in atopic dermatitis,
trials can settle this issue. 40,41 probably due to antiallergic properties of
tea polyphenols. 45
d. Special silk fabric
As it is well known, irritant factors
aggravate childhood atopic dermatitis. A
144
h. Natural honey and beeswax 3. Jenner N, Campbell J, Marks R.

A mixture of honey, olive oil and Morbidity and cost of atopic
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39. Koo J, Arian S. Traditional dermatitis or psoriasis: partially
Chinese Medicine for the controlled, single-blinded study.
treatment of dermatologic Complement Ther Med 2003; 11:
disorders. Arch Dermatol 1998; 226-34.
134: 1388-93. 47. Shainhouse T, Eichenfield LF.
40. Smolle J. Homeopathy in Long-term safety of tacrolimus
dermatology. Dermatol Ther ointment in children treated for
2003; 16: 93-7. atopic dermatitis. Expert Opin
41. Itamura R, Hosoya R. Drug Saf 2003; 2: 457-65.
Homeopathic treatment of
147
Biostatistics: Introduction…. Tariq Zaman and Abbas Raza
Stat Corner
Biostatistics - I: Introduction, role &
applications in medicine
Tariq Zaman, Abbas Raza*
Dermatology department, Allama Iqbal Medical College, Lahore.
* Department of Medicine, King Edward Medical College, Lahore.
Understanding the fundamentals of biostatistics is essential for every clinician in order

to plan a scientific study and interpret its results . Unfortunately, this area had been
neglected at the level of undergraduate and postgraduate medical education in country.
The terms like X2 test, student t test, p value, sensitivity, specificity etc. are very scary
for clinicians and while going through a medical journal, the pages describing the
statistical methods are generally skipped. As a part of CME activity for its readers,
JPAD has started a series of articles covering this dry and boring topic. The authors
have simplified the subject in a reader-friendly manner so that it is easy to comprehend
and assimilate. (Editor).
“The fundamental gospel of statistics is to statistical conclusions. This article is

push back the domain of ignorance, intended to give an introduction to
prejudice, rule-of-thumb, arbitrary or biostatistics, its evolution through time
premature decisions, tradition, and and its role and applications in health
dogmatism and to increase the domain in sciences.
which decisions are made and principles
are formulated on the basis of analyzed Historical perspective
quantitative facts.”….Robert W. Burgess From dawn of civilization to about AD
1000, medical treatment was directed
Statistics is deeply engraved in our daily against supposed etiologies, e.g., evil
life e.g. the chances of being successful in spirits, draining bad blood, etc. The first
a particular assignment or examination, ever mention of statistical probabilities of
and at a wider scale, success of a particular an outcome is found in Talmud (AD 300).
plan (e.g. healthcare) in a community or Hindu culture takes the credit of using
winning a war against enemy. All these numerical and decimal system, the
issues are generally judged qualitatively. foundation of statistics. Arabic
Calculating the risks, benefits and mathematician, Khowarizmi, defined rules
probabilities involved in mathematical for adding, substracting, multiplying and
terms is statistics. dividing (AD 800). Huygen and John
Grant (1660) advanced this knowledge
Present series of articles, which will further.
regularly appear in successive issues of the
journal, is written to make reader more Twentieth century saw an explosion of
effective in interpretation of research medical technology and treatment in the
literature in medicine and be able to design fields of surgery, medic ine and their
research study and draw appropriate subspecialties. It became increasingly
necessary to standardize medical practice
Dr. Tariq Zaman,
210 G.T.Road, Baghban pura, Lahore.
148
on sound scientific basis, and interpret the e.g. statistics regarding economics,
controversies in the treatment of similar business, industry, education, health,
conditions from growing body of diseases, etc. They are collected from
evidence. In the mid-twentieth century, records, surveys or experiments.
“Case Study” and “Case Series” were a
common way to prove that a treatment was Secondly, the term statistics is used to
beneficial. Most research work done was refer a body of knowledge, a discipline or
anecdotal than scientific. James Lind, a a branch of science. It is a science of
Royal Navy surgeon, carried out first figures. It deals with the principles and
recorded randomized clinical trial in 1747, methods for collection, processing,
applying six dif ferent treatments for analysis, presentation and interpretation of
scurvy cure in sailors. It took 200 years numerical data. It is an inferential science
before randomized controlled trials too, and helps us to draw reliable and valid
became the standard for evaluation of inferences from the available data
various therapeutic options. particularly in the fields of
experimentation and research. It is a
Sir Ronald Fisher and Austin Bradford are science of decision-making, and is the
remembered as fathers of modern only way out to take decisions in the face
statistics. They published a series of of uncertainty.
articles in “Lancet” and “British Medical
Journal” (1937-1947)), reporting their The term “biostatistics” is used for the
randomized trials e.g. streptomycin vs. statistics related to the biological sciences
placebo in lung tuberculosis, and stressing like biology, medicine or public health. So
upon introduction of biostatistics in biostatistics refers either to the data
medical curricula. Archic Chocrane, a arising out of the biological sciences or to
British general practitioner started current the science that deals with the application
movement of systematic statistical reviews of statistical methods to the data derived
of available evidence in medicine, thus from these fields.
laying the foundation of Evidence Based
Medicine and Practice. Statistical methods fall into two broad
categories, descriptive statistics and
Statistics and biostatistics inferential statistics.
The word “statistics” has originated from Descriptive Statistics

Latin words status (meaning ‘state') or This branch of statistics deals with the
statisticus (meaning ‘state affairs’) and has summarization and description of data. It
been used for the information useful for is the most basic form of statistics that lays
the state, e.g. information about size of the foundation for all statistical
population or armed forces, about amount knowledge. The methods of descriptive
of revenue, reserves or salaries, etc. etc. statistics are used to consolidate the data in
But now the term ‘statistics’ is used in two the form of tables, charts or graphs. They
different contexts. are also applied to compute the numerical
quantities that provide information about
Firstly, it is used as a synonym of data and the central tendency and spread of the
refers to systematically arranged data, e.g. mean, median, mode, standard
numerical facts and figures of any kind, deviation, variance, etc.
149
Inferential Statistics to develop critical attitude towards

It is the branch of statistics that deals with research data and inferences presented at
making inferences from the collected data. any forum or published in any scientific
The methods of inferential statistics are journal, newspaper or magazine. The
applied to test statistical hypothesis and clinicians also require some working
draw conclusions by computing knowledge of basic statistical techniques
confidence interval or statistical while evaluating clinical features,
significance. They are used to estimate diagnosis, prognosis and management of
population parameters using data obtained their patients. In fact, neither medical
from a sample from that population. practice nor research can be properly
planned, executed or assessed without
Role of statistics in medicine understanding the fundamentals of
Statistical analysis is a fundamental biostatistics.
component of modern research in all
disciplines of science, and medicine is no Application and uses of statistics in
exception. In medical research the role of health sciences
statistics starts from the planning phase in “A knowledge of statistics is like a
deciding study design, selecting sampling knowledge of foreign languages or of
technique and formulating research algebra; it may prove of use at any time
methodology. The statistical tools have under any circumstances.”…Arthur L.
their application in data collection and its Bowley
handling, analysis and interpretation.
Statistical treatment of data is required to The biostatistics has its application and
draw valid conclusions, which have to be uses in the following broad areas of basic
evaluated in the light of statistical logic. medical sciences, applied medicine and
Statistical methods are also helpful for public health:
comprehensible and effective presentation
of research data and results. In addition, • In def ining the normal of various
clear concepts of basic principles of body characteristic and their limits
biostatistics are also required to (range of normality) in a
understand the data presented by others. population, i.e. at what point these
measures become ‘abnormal’ or
“A knowledge of statistical methods is not ‘pathological’. For example,
only essential for those who present normal value and range of blood
statistical arguments it is also needed by pressure, heart rate, weight,
those on the receiving end.” …R.G.D. height, blood counts, serum
Allen biochemistry, etc.
• In finding the difference between
Various statistical tools are extensively means and proportions of normal
used in medical journals for description of variables (body characteristics) in
data and for drawing conclusions from two samples with different
research findings. Knowledge of statistical parameters or in the same sample
methods is necessary to assess the validity at two places or in two different
of evidence in these articles. periods, e.g. difference between
Understanding of principles and concepts means of weight or height in
of biostatistics is an essential prerequisite various age, sex, racial or ethnic
150
groups, or difference between contaminated water in diarrhea or

heart rate or blood pressure at dysentery by comparing the
basal level and after exercise in proportions of disease attacks in
the same sample. groups of individuals drinking
• In finding the correlation between boiled or unboiled water.
two variables. Whether one Similarly, evaluation of the role of
var iable increases or decreases smoking in lung cancer or high
proportionately with the other cholesterol diet in ischemic heart
variable, and if so by how much. disease, etc.
For example, correlation between • In determining the usefulness of
age and weight, or between height various public health programs.
and weight, etc. For example, evaluation of
• In determining the action, dosage vaccination for a particular
and potency of a new drug in infection by comparing the
pharmacological studies. By proportions of attacks/deaths in
statistical techniques the action of vaccinated and unvaccinated
a drug is compared in animals or subjects and by analyzing the
human volunteers with a placebo statistical significance of the
to determine whether it is due to difference observed.
the drug or by chance. Or the • In collecting the demographic,
action of the new drug is health and vital statistics, which
compared with a standard drug or are important indicators of health
with various doses. status of the community. They
• In identifying and documenting have a pivotal role in formulating
the clinical features of diseases, health policy and in making future
e.g. signs, symptoms, course, health plans.
prognosis, complications, etc. and
their variability among various In addition, application of statistical
groups of patients or their principles is necessary in many other areas
association with various of medicine and public health, like
characteristics like age, sex, etc. construction of life tables and survival rate
• In analyzing the sensitivity and calculation, risk specification, cost
specificity of various laboratory analysis, quality of life indices, etc.
tests, clinical procedures or
diagnostic criteria. Latest trends in statistics
• In planning and conduct of Preceding decade has seen following
clinical trials in a particular developments in the clinical application of
disease for efficacy and safety of a biostatistics in health care sciences:
drug, surgical procedure, radiation
or physiotherapy, etc. Evidence Based Medicine (EBM)
• In evaluating the role of basic Evidence Based Medicine has been
underlying factors in the causation defined as the “the conscientious, explicit
of different ailments in the and judicious use of best available
epidemiological studies. For evidence in making decisions about cases
example, determining the role of of individual patients”. It was realized in
1980s, that there were large variations in
151
the treatment strategies offered to patients the non-statisticians including health

with similar ailments. For example, rates professionals. Statistical analysis of any
of prostatectomy (for benign hyperplasia), study is always considered to be the job of
hysterectomy (for menorrghagia) and statistician. Computers and commercial
cataract surgery varied 2.5, 3.0 and 20 statistical software packages have not only
times respectively. Evidence based allayed the fear of mathematical
medicine started with the appreciation of calculations but also offer economy of
the need for uniformity of treatment for time. Research worker can now apply the
same conditions. This uniformity is based appropriate statistical methods to his
upon statistical analysis of randomized research work himself with the help of
controlled trials on the same subject. these softwares. However, a sound
knowledge of basic principles of
Meta-analysis biostatistics still remains mandatory to use
Meta-analysis forms the basis of EBM. It these packages.
involves inclusion of multiple studies
according to preset inclusion and Conclusions
exclusion criteria in which the results from
The medical students, clinicians or
all the studies are pooled and analyzed
research workers should not depend solely
statistically as if it were from one large on the statistician for statistical analysis.
study. The meta-analysis, also called as They should learn the basic principles of
“Systematic Literature Review” explains
biostatistics and should apply these
the results quantit atively. In contrast the
methods correctly for unprejudiced
traditional “Literature Review” examines assessment and management of the
the relevant literature for general trends patients, for proper planning of scientific
and patterns in a particular subject in
studies, for reinforcement of their own
qualitative terms only.
research conclusions and for critical
evaluation of the work done by others.
Guidelines
EBM clarifies one particular facet of
disease management (e.g. thrombolysis in Further reading
acute myocardial infarction). 1. Bluman AG, ed. Elementary Statistics
– step by step approach, 5th edn.
Comprehensive disease management Philadelphia : McGraw -Hill; 2004.
requires treatment according to the best 2. Coogan D, ed. Statistics in clinical
available evidence at each step till medicine, 2 nd edn. London: BMJ
Publications; 1999.
recovery. A disease may also not follow 3. Gaddis G, Gaddis M. Introduction to
the typical pattern. Guidelines are biostatistics; part II, the descriptive
formulated by a panel of experts of the statistics. Ann Emerg Med 1990; 19:
309-15.
particular disease. Literature is searched 4. Krishnamurty GB, Kasovia-Schmitt
for each step of disease management, P, Ostroff DJ, eds. Statistics – An
strength of evidence is judged, and a interactive text for health and life
sciences. London: Jones & Bartlett
consensus is reached upon before Publishers International; 1995.
publication of guidelines. 5. Mann PS, editor. Introductory
Statistics, 5th edn . Singapore: John
Willy & Sons; 2004.
Role of computer in statistics
Mathematical calculations used in
biostatistics have always been feared by
152
Case report
Dyskeratosis congenita (DC) in a Saudi
boy: an uncommon genodermatosis
A. Y. Saadeldin, Satti A. Satti, Ali S. Dammas
Pediatric & Neonatology Department, King Fahad Hospital at Al-Baha, Al-Baha,
Saudi Arabia.
Abstract A 6-year-old Saudi boy presented to our hospital with severe thrombocytopenia. The
patient was managed for a long time (6 years) as having chronic idiopathic
thrombocytopenic purpura. Later on features consistent with dyskeratosis congenita
were recognized by the authors. The main features were: skin manifestations, nail
dystrophy, alopecia totalis, microcephaly and mental retardation. The condition was
associated with acute necrotizing ulcerative gingivitis. At the age of 10, he developed
pancytopenia and died at the age of 14 years from acute fulminant sepsis.
Key words
Dyskeratosis congenita, acute necrotizing ulcerative gingivitis, pancytopenia.
have progressive bone marrow (BM)

Introduction failure, which is the major cause of death. 6
Few of the patients die from pulmonary
Dyskeratosis congenital (DC) is a rare complications (e.g. fibrosis), and
genodermatosis and multisystem disorder, malignancy (acute myeloid leukemia and
characterized by cutaneous reticulated Hodgkin disease) . 7
hyperpigmentation, nail dystrophy and
mucous membrane les ions (ulcers, Case report
premalignant oral leucoplakia), and
progressive pancytopenia. 1 Bone marrow At the age of 6 years a Saudi boy
hypoplasia is frequent. 2 The majority of presented to our hospital complaining of
cases (90%) are males. 3 The age of the following since the age of 6 months:
presentation is between 5-15 years.4 The chronic recurrent bleeding from gums,
most common pattern of inheritance is X- easy bruising following minor injuries,
linked recessive caused by mutations in epistaxis and excessive tearing. During
the DKC1 gene. 1 Autosomal dominant and pregnancy his mother developed chicken
recessive forms exist. The gene causing pox. Delivery was normal with no
the X-linked recessive forms is located to problems. Developmental history revealed
Xq 28 and codes dyskerin, a 514 amino delayed speech and also other milestones.
acid protein. 5 80% of patients with DC Parents were first degree cousins. There
were nine siblings (7 males and 2 females)
Dr. Imad Yassin Saadeldin Consultant all were normal except one brother who
Pediatrician, King Fahad Hospital at Al-Baha, Al- died at the age of 3 years with similar
Baha, PO Box 204, Al-Baha, Saudi Arabia.
Tel.(Mobile):+966-53777041, Work: +966-7-
complaint and physical picture as our
725-4000 ext.-3730/3721 patient and another 17-year-old brother
Fax:+966-725-4184/725-2188
with cutaneous hyperpigmentation, nail
E-mail: isaadeldin@yahoo.com
dystrophy and alopecia totalis but no
153
Dyskeratosis congenital in a Saudi boy … A. Y. Saadeldin et al.
hematological manifestations. There were

seven maternal uncles who died in early
childhood (the cause not known).
Physical examination Our patient was

pale, microcephalic, with alopecia totalis
and sparse hair of eyebrows and eye lashes
(Figure 1), dystrophic nails of the hands Figure 1 Pale, microcephalic child with
diffuse alopecia over scalp and sparse
(Figure 2) and feet, reticular skin with eyebrows and eye lashes
hyperpigmentation of the neck (Figure 3),
upper chest and lower limbs, multiple
purpuric spots with ecchymosis over the
neck and bleeding spots in the gums,
throat and uvula. The skin on extensor
surfaces of upper and lower limbs and
soles was hyperker atotic. There were also
subconjunctival haemorrhages (Figure 4).
Dental caries with enamel hypoplasia was Figure 2 Dystrophic fingernails
evident. Mouth examination showed:
acute necrotizing ulcerative gingivitis
(ANUG) with focal acute
pseudomembranous candidiasis. No
hepatosplenomegaly or lymphadenopathy
detected, with normal joints and genitalia.
Vital signs were normal. The weight,
height and head circumference were all
Figure 3 Reticulate hyperpigmentation
below the 3rd centile. Retinal hemorrhages affecting the sides of neck.
in both eyes revealed by fundoscopy.
Investigations done on admission: 1. CBC:

WBC: 8.6x109/l, HB: 7.2 g/dl, MCV:
100.0 fl, MCH: 31.2 pg, MCHC: 31.2 g/dl,
RDW: 17.9 between 2-17x109/l,
reticulocyte count of 1.6%. 2. Peripheral
smear: macrocytic anemia with severe
thrombocytopenia. 3. Prothrombin time
Figure 3 Subconjunctival hemorrhage in the
(PT) and activated partial thromboplastin
right eye.
time (APTT): normal. 4. Chemistry was
normal. fever, multiple oral ulceration and same
previous physical changes. Investigations
Later on the patient had frequent
at this time revealed: 1. CBC: severe
admissions in the pediatric department
pancytopenia, ESR: 122mm/hr. 2.
with severe anemia, severe Peripheral smear: anisocytosis,
thrombocytopenia and bleeding, mainly normochromia, some macrocytes and few
from gums. Lastly he presented in August,
spherocytes. There was severe leucopenia
2000 (at the age of 12 years) with high
154
with 51% lymphocytes and very low pediatricians of the importance of knowing
platelets count. 3. PT and APTT: still such a rare condition like DC. We saw the
normal 4. Chemistry, urea and electrolytes patient for the fir st time when he was
and hepatitis panel were normal 5. Hb referred to our hospital at the age of 10
electrophoresis: HbA-97.9%, HbA2-2.1% years, due to pancytopenia. At that time
6. Chest X-ray and X-ray of limbs were the following features were recognized:
normal. 7. Ultrasound (U/S) abdomen microcephaly, alopecia totalis with sparse
showed: coarse echo pattern of the liver. 8. hair of eye lashes and brows, dystrophic
Liver function tests were mildly disturbed. nails of the hands and feet, dermatologic
9. High serum ferritin of 3216 ng/ml. 10. features such as reticular hyperkeratotic
Bone marrow aspirate: showed a picture of skin and hyperpigmentation, which is the
aplastic anemia. 11. Septic screening was usual presentation in DC. 1 He also
done many times, showed no bacterial presented with mucous membrane changes
growth. CT brain: showed mild cerebral in the form of acute necrotizing ulcerative
atrophy. gingivitis (ANUG), which from literature
review is found to be common in patients
Management The patient received many with immunodeficiency and HIV
courses of prednisolone tablets since the 8
infection. ANUG is rare in DC. The
age of 6 years in different hospitals before patient was found to have bone marrow
he was seen by the authors. He received failure, which is considered to be a late
also short courses of androgens: manifestation of DC. 2,9,10 Differential
oxymethalone 60mg OD and androlone diagnosis to be considered is Hoyeraal-
25mg intramuscular every 2 weeks. Hreidarsson syndrome, a severe infantile
During the last 8 years, our patient variant of DC, inherited as X-linked
received many packed red blood cells and manner, in which there may be overlap
platelets transfusions due to severe anemia with DC. 1,11,12 The features of this
and thrombocytopenia, and many courses syndrome include: intrauterine growth
of parenteral and oral antibiotics, due to retardation, microcephaly, mental
repeated systemic infections of the lower retardation, cerebral malformation,
respiratory tract. Bone marrow immunodeficiency and progressive bone
transplantation was planned, but at the age marrow failure. 1 Our patient presented
of 14 years (in 2002) our patient presented with microcephaly, mental retardation and
with severe fulminant sepsis and in spite brain atrophy, which is not the usual
of extensive and aggressive management presentation of classical DC. 12
in our pediatric intensive care unit he
expired after two days. Other differential diagnoses are
Rothmund-Thomson syndrome (short
Discussion stature, skin telangiectasias, small hands
and feet, hypoplastic thumbs with a high
Since the age of 6 years our patient had risk of osteosarcoma) and graft versus host
been admitted in the hospital due to disease.4 Our patient died at the age of 14
bleeding per gums and skin and found to years with fulminant sepsis which is one
have severe thrombocytopenia. He was of the leading causes of death in DC. 13
diagnosed and managed as having Other causes of death include bone
idiopathic thrombocytop enic purpura. This marrow failure6 and the development of
attracts the attention to physicians and malignancies, especially acute myeloid
155
Dyskeratosis congenital in a Saudi boy … A. Y. Saadeldin et al.
leukemia14 and squamous cell carcinoma, 2 6. Dokal I. Dyskeratosis congenita. Br. J

which can occur on leucoplakia or normal Haematol 1999; 105: S11-5.
7. Baykal C, Buyukbabani N, Kavak A.
mucous membranes and skin. 7 As Dyskeratosis congenita associated
mentioned, there was one brother, who with Hodgkin disease. Eur J
died at the age of 3 years, another 17 year- Dermatol 1998;.8: 385-7.
8. SC. HIV infection and periodontal
old brother, who is still living, both with disease. Ann R Australs Coll Dent
similar features and 7 maternal uncles who Surg 2000; 15: 331-4.
died undiagnosed, in early childhood, 9. Forni GL, Melevendi C, Japelli S,
Rosore-Quortino A. Dyskeratosis
suggesting an X-linked mode of congenita: unusual presenting features
inheritance. The patient received within kindred. Ped Hematol Oncol
androgens for the bone marrow failure as 1993; 10: 145-9.
10. Putterman C, Safadi R, Zlotogora J et
there is about 50% response15 in many al. Treatment of the manifestation of
cases, but in our patient there was no dyskeratosis congenita. Ann Hematol
improvement. 1993; 66: 209-12.
11. Cossu F, Vulliamy TJ, Marrone A et
al. A novel DKC1 mutation, severe
References combined immunodeficiency, and
bone marrow transplantation in an
1. Yagamahi R, Kimyai-Asadi A, infant with Hoyeraal-Hreidarsson
Rostamiani K et al. Overlap of syndrome. Br J Ha ematol 2002; 119:
dyskeratosis congenita with the 765-8.
Hoyeraal-Hreidarsson syndrome. J 12. Knight SW, Heiss NS, Vulliamy TJ:
Pediatr 2000; 136 : 390-3. Unexplained aplastic anaemia,
2. Jacobs P, Saxe N, Gordon W, Nelson immunodeficiency, and cerebellar
M. Dyskeratosis congenita: hypoplasia (Hoyeraal-Hreidarsson)
haematologic, cytogenetic, and due to mutations in the dyskeratosis
dermatologic studies. Scand J congenita gene, DKC1. Br J
Haematol 1984; 32: 461-8. Haematol 1999; 107: 335-9.
3. McKusick VA, ed. Mandelian 13. Davidson HR, Connor JM.
inheritance in man, 11th edn. Dyskeratosis congenita. J Med Genet
Baltimore : Johns Hopkins; 1994. 1988; 25: 843-6.
4. Chan EF. Dyskeratosis congenita. 14. Knight S, Vulliamy T, Copplestone A
Nov.5.2001; eMedicine. et al. Dyskeratosis congenita (DC)
5. Connor JM, Gatherer D, Gray FC et registry: identification of new features
al. Assignment of the gene for of DC. Br J Haematol 1998; 103:
dyskeratosis congenita to Xq28. Hum 990-6.
Genet 1986; 72: 348-51.
156
Case report
Nevoid psoriasis: an uncommon
blaschkolinear dermatosis
Arfan ul Bari, Simeen Ber Rahman*
Consultant Dermatologist, PAF Hospital, Sargodha

* Dermatology Department, Military Hospital, Rawalpindi
Abstract Nevoid or linear psoriasis is an uncommon form of psoriasis characterized by the

linear or zosteriform distribution of the psoriatic lesions. It usually follows the lines of
Blaschko with unilateral involvement. It is commonly confused clinically and
histopathologically with verrucous epidermal nevus. We report a young male, who had
linear psoriasis in a unilateral distribution over his shoulder and arm. He was
diagnosed on clinical as well as histological ground and was managed on the lines of
psoriasis.
Key words
Linear psoriasis, nevoid psoriasis, lines of Blaschko, inflammatory linear verrucous
epidermal naevi.
blaschkolinear distribution mostly occur

Introduction together with scattered lesions, but
occasionally they may be isolated and may
Psoriasis is a common chronic , genetic, present in a purely nevoid form following
noncontagious inflammatory skin disorder Blaschko’s lines, may koebnerize, or may
that appears in many clinico- superimpose an epidermal nevus.7,8 Very
morphological forms and can affect any rarely it may coexist with another rare
part of the body. Nevoid or linear psoriasis dermatosis that follows Blaschko's line,
is a rare variant of this common disorder e.g. porokeratotic eccrine ostial and
and is characterized by a linear dermal duct nevus. 9 Several clinical
distribution of the psoriatic lesions. It entities resemble nevoid psoriasis, and
follows the lines of Blaschko with differential diagnosis is important. It is the
unilateral involvement and usually occurs inflammatory linear verrucous nevus
in young adults but has also been reported (ILVEN) that is most commonly confused
in childhood. 1,2 The pathogenesis is with linear psoriasis and a clear distinction
unclear, but it could be explained as a between linear psoriasis vulgaris and
result of the migration of cells harboring a (ILVEN) can be difficult because of the
somatic mutation following the lines of clinical and histopathologic similarities of
Blaschko during early embryogenesis . 3,4 It the two conditions . 10 An association of
was first described in 1951 by Leslie5 and ILVEN with psoriasis vulgaris has also
Sobel. 6 In psoriasis lesions in a been reported. 7 Lesions of the ILVEN are
pruritic, most commonly occur on the legs,
Squadron Leader Dr. Arfan ul Bari, pelvis, and buttock, have early age of
Consultant Dermatologist, PAF Hospital, onset and female predominance. The
Sargodha
Ph# (off): 0451-5553307, (res): 0451-5553308 congenital hemidysplasia with
E mail: albariul@yahoo.com ichthyosiform nevus and limb deficiency
157
Nevoid psoriasis: an uncommon blaschko linear dermatosis Arfan ul Bari and Simeen Ber Rahman
(CHILD) syndrome presents with

verrucous plaques in a segmental
distribution but other associated features
clearly differentiate it. Lichen striatus and
linear lichen planus also resemble nevoid
psoriasis or ILVEN, however, their
histology is quite distinct. 10-12 Above
clinical and histopathologic features
clearly differentiate these disorders from
linear psoriasis. In difficult to diagnose
cases, involucrin immunohistochemistry
can be a useful diagnostic tool to confirm Figure 1 Linear erythematous , scaly eruption
diagnosis of nevoid psoriasis . 13 Treatment affecting left axilla, shoulder ad upper arm.
with keratolytics and topical calcipotriol,
topical steroids and other topical anti-
psoriatic regimens lead to a s ignificant
improvement. 1,2 The purpose of reporting
the case was to highlight the existence of
nevoid or linear psoriasis as an entity.
Case report
A 25-year -old man, presented with a

slightly pruritic, linear plaques over his
left shoulder and adjacent arm. The lesions Figure 2 Another view of linear
started appearing three years ago as small hyperkeratotic, erythematous and scaly
discrete papules and plaques roughly in a eruption.
linear fashion on back of his right shoulder
and arm and gradually progressed and
coalesced to form large linear scaly
plaques. He had no relevant personal or
family history. On clinical examination,
large erythematous scaly verrucous lesions
were seen that were linearly distributed
over back of his left shoulder axilla and
outer aspect of the left upper arm (Figure
1 and 2). There were no lesions suggestive
Figure 3 Histopathology of the lesion,
of psoriasis else where over his skin nails
psoriasiform hyperplasia of epidermis along
or scalp. Skin biopsy revealed classical with Munro’s microabscesses.
histological features of psoriasis showing
acanthosis with regular elongation of rete alternating with columns of agranulosis
ridges, parakeratosis, Munro's with parakeratosis as we see in ILVEN.
microabscesses, spongiform pustules
(Figure 3). There were no columns of Clinical examination and results of
hypergranulosis with orthokeratosis histopathology confirmed the diagnosis of
unilateral nevoid or linear psoriasis. He
158
was managed with topical anti-psoriatic ILVEN. The criteria for the diagnosis of
ointment (containing tar, salicylic acid, ILVEN established by Altman and
topical steroid dispensed in emulsifying Mehregan14 include early age of onset, 4
ointment). times more common among women than
men, frequent involvement of the left leg,
Discussion pruritus, psoriasiform appearance,
persistence, and resistance to treatment.
Psoriasis is one of a number of Among these criteria, a differential point is
autoimmune diseases that display that the lesions of ILVEN take the form of
significant HLA associations (HLA-Cw6). intensely pruritic linear groups of
However, only about 10% of Cw6-positive excoriated eczematous papules, which
individuals develop disease, suggesting proves extremely refractory to therapy.
that other genetic and/or environmental The histopathologic aspect of ILVEN is
factors must be involved. Several very similar to psoriasis, but is
compelling lines of epidemiologic characterized by columns of
evidence indicate that psoriasis hypergranulosis with orthokeratosis
susceptibility is inherited, albeit not in a alternating with columns of agranulosis
simple monogenic fashion, and that with parakeratosis. The case reported by
genetic, rather than environmental, factors us was clinically more suggestive of
are primarily responsible for the variability nevoid psoriasis rather than ILVEN, as the
in inheritance of psoriasis. Taken together, patient was male, the disease had late
these observations suggest that one or onset, and the lesions were not much
more loci in addition to HLA are pruritic and were distributed over upper
necessary for the development of half of the body (these features were
psoriasis. The number of additional loci is against ILVEN). Moreover, the histology
likely to be small, because i) the disease is of the lesion revealed classical features of
very common ii) substantial excess risk of psoriasis and there were no alternating
psoriasis is observed in first degree columns of hypergranulosis with
relatives, and iii) nevoid variants of orthokeratosis and agranulosis with
psoriasis have been reported, suggestive of parakeratosis. The response to anti-
somatic mutation of a single gene during psoriatic treatment was satisfactory.
development. 3,4 The existence of a linear
psoriasis has frequently been a subject of Conclusion
debate. Several authors have disputed the
existence of true line ar psoriasis and Psoriasis may koebnerize in a linear
contend that many reports of linear fashion, may superimpose or invade an
psoriasis represent either inflammatory epidermal nevus but a nevoid form
linear verrucous epidermal nevus (ILVEN) following Blaschko’s lines should be
in a patient with psoriasis, a nevus, or even taken a separate entity.
an invasion of ILVEN a by psoriasis as a
manifestation of the isomorphic References
reaction. 10,11
In contrast, some authors have 1. Atherton DJ, Kahana M, Russel-Jones
R. Naevoid psoriasis. Br J Dermatol
a strong opinion that linear psoriasis is a 1989; 120 : 837-41.
separate entity. Therefore, before 2. Saraswat A, Sandhu K, Shukla R,
diagnosis, it should be differentiated Handa S. Unilateral linear psoriasis
with palmoplantar, nail, and scalp
clinically and histopathologically from
159
Nevoid psoriasis: an uncommon blaschko linear dermatosis Arfan ul Bari and Simeen Ber Rahman
involvement. Pediatr Dermatol 2004; 10. de Jong E, Rulo H, van de Kerkhof

21: 70-3. PC. Inflammatory linear verrucous
3. Elder JT, Henseler T, Christophers E epidermal nevus (ILVEN) versus
et al. Of genes and antigens: the linear psoriasis: a clinical, histological
inheritance of psoriasis. J Invest and immunohistochemical study. Acta
Dermatol 1994; 103: 150S-153S. Derm Venereol 1991; 71: 343-6.
4. Happle R. Somatic recombination 11. Lee SH, Rogers M. Inflammatory
may explain linear psoriasis. J Med linear verrucous epidermal naevi: a
Genet 1991; 28: 337. review of 23 cases. Austral J
5. Leslie G. Linear psoriasis. Br J Dermatol 2001; 42: 252-6.
Dermatol 1951; 63: 262-263. 12. Grosshans EM. Acquired
6. Sobel M. Linear psoriasis. Arch blaschkolinear dermatoses. Am J Med
Dermatol Syph 1951; 63: 267-71. Genet 1999 6; 85: 334 -7
7. Menni S, Restano L, Gianotti R, 13. Ginarte M, Fernandez-Redondo V,
Boccardi D. Inflammatory linear Toriboi J. Unilateral psoriasis: a case
verrucous epidermal nevus (ILVEN) individualized by means of
and psoriasis in a child?. Int J involucrin. Cutis 2000; 65: 167-70.
Dermatol 2000; 39: 30-2. 14. Altman J, Mehregan AH.
8. Bondi EE. Psoriasis overlying an Inflammatory linear verrucous
epidermal nevus. Arch Dermatol epidermal nevus. Arch Dermatol
1979; 115 : 624-5. 1971; 104 : 385-9.
9. Yu HJ, Ko JY, Kwon HM, Kim JS.
Linear psoriasis with porokeratotic
eccrine ostial and dermal duct nevus.
J Am Acad Dermatol 2004; 50: S81-3.
160
Quiz
Generalized pustular rash of acute onset
Asher Ahmed Mashood
Consultant Dermatologist, Combin ed Military Hospital, Peshawar
Report of a case
A 23-year-old married lady reported in

skin OPD with a generalized body rash
and low-grade fever of 2 days duration.
The patient suffered from renal colic three
days prior to the development of the rash
for which she received oral ampicillin.
On examination, the skin of the whole

body was erythematous. Over the
background of erythema, there were
multiple, superficial, pinpoint pustules,
which were discrete and at places
coalescing into sheets of pus (Figure 1).
Microscopic findings
The histopathological examination of the

skin specimen revealed pustules within the
Figure 1 Multiple discrete and coalescing
stratum corneum. The epidermis showed pustules over a background of diffuse
acanthosis and spon giosis. The dermis erythema. Dried squames are seen at places.
showed mild edema and occasional
extravas ation of neutrophils and
eosinophils.

Dr. Asher Ahmed Mashood,
Consultant Dermatologist CMH, Peshawar
Ph# 091 2016154, 091 271779
E-mail: asherahmed67@yahoo.com
161
Generalized pustular rash of acute onset Asher A. Mashood
Diagnosis References
Acute exanthemic pustulosis 1. Manders SM, Heymann WR. Acute

generalized exanthemic pustulosis.
Cutis 1994; 54: 194-6.
Discussion 2. Jay S, Kang J, Watcher MA et al.
Localized pustular skin eruption.
Acute exanthemic pustulosis is an eruption Localized pustular drug eruption
secondary to ampicillin. Arch
of generalized pustular rash over an Dermatol 1994; 130: 787 -90.
erythematous background, all over the 3. Armster H, Schwarz T.
body especially the trunk. 1 The rash occurs Arzneimittelreaktion auf Amoxicillin
unter dem blid eines toxischen
within 24-48 hours of the administration pustuloderms. Hautarzt 1991; 42:
of the offending drug. The pustules are 713-6.
monomorphic, superficial and diffuse. The 4. Gebhardt M, Lusting A, Bocker T et
al. Acute generalized exanthemic
rash usually starts with fever and settles pustulosis (AGEP): manifestation of
spontaneously with desquamation. Facial drug allergy to propicillin. Contact
oedema, purpura, vesicles, blisters and Dermatitis 1995; 33: 204-5.
5. Kalb RE, Grossman ME. Pustular
erythema-multiforme like lesions may also eruption following administration of
be seen. The drugs implicated are cephadrine. Cutis 1986; 38: 58-60.
ampicillin, 2 amoxycillin, 3 propicillin, 4 6. Jackson H, Vion B, Levy PM.
Generalized pustular drug rash due to
cephadrine, 5 cephalexin, 6 cotrimoxazole, 7 cephalexin. Dermatologica 1988;
doxycycline,8 chloramphenicol, 177 : 292-4.
norfloxacin, ofloxacin, streptomycin , 11
9 10 7. MacDonald KJS, Green CK, Kenicer
KJA. Pustular dermatosis induced by
salazopyrine, 12 pyrimethamine, frusamide, co-trimoxazole. Br Med J 1986; 293:
nitrazepam, itraconazole, 1 diltiazem, 13 1279-80.
captopril, 14 enalapril,15 acetylsalicylic 8. Trueb RM, Burg G. Acute
generalized exanthemic pustulosis
acid,16 naproxen, 17 allopurinol, 18
due to doxycycline. Dermatology
hydroxychloroquine,19 chlorpromazine, 20 1993; 186 : 75-8.
phenytoin, 21 and carbamezapine. 22 9. Shelley ED, Shelly WB. The
subcorneal pustular eruption; an
example induced by norfloxacin.
The main differential diagnosis is pustular Cutis 1988; 42: 24-7.
psoriasis. 1,23 It can be differentiated from 10. Tsuada S, Kato K, Karasima T et al.
acute exanthemic pustulosis on both Toxic pustuloderma induced by
ofloxacin. Acta Derm Venereol
clinical and histological basis. The (Stockh) 1993; 73: 382-4.
histopat hological picture is identical to 11. Kushimoto H, Aoki T. Toxic
subcorneal pustular dermatosis, erythema with generalized follicular
pustules caused by streptomycin.
characterized by subcorneal pustules and Arch Dermatol 1981; 117 : 444-5.
mixed dermal infiltrate composed of 12. Gallais V, Grange F, De Bandt M et
neutrophils and eosinophils. Since it is a al. Toxidermie a la
salazosulfapyridine. Erythrodermie
self-limiting disease, general measures like pustuleuse et syndrome
fluid and electrolyte balance, bladder care, psedolympomateux: 2 observations.
prevention of infection, pain-killers, anti- Ann Dermatol Venereol 1994; 121:
11-4.
pruritic tablets and some bland cream or 13. Lambert DG, Dalac S, Beer F et al.
lotion to reduce the discomfort are Acute generalized exanthemic
generally sufficient. pustular dermatitis induced by
diltiazem. Br J Dermatol 1988; 118:
308-9.
162
14. Carroll J, Thaler M, Grossman E et Derm Venereol (Stockh) 1990; 70:

al. Generalized pustular eruption 250-1.
associated with converting enzyme 20. Burrows NP, Ratnavel RC, Norris
inhibitor therapy. Cutis 1995; 56: PG. Pustular eruptions after
276-8. chlorpromazine. Br Med J 1994; 309 :
15. Ferguson JE, Chalmers RJ. Enalapril- 97.
induced toxic pustuloderma. Clin Exp 21. Kleier RS, Breneman DL, Boiko S.
Dermatol 1996; 21: 54-5. Generalized pustulation as a
16. Ballmer-Weber BK, Windmer M, manifestation of the anticonvulsant
Burg G. Acetylsalicylsaure-induzierte hypersensitivity syndrome. Arch
generalisierte pustulose. Schweiz Med Dermatol 1991; 127: 1361-4.
Wochenschr 1993; 123: 542-6. 22. Commens CA, Fisher GO. Toxic
17. Grattan CEH. Generalized pustular pustuloderma following
drug rash due to naproxen. carbamazepine therapy. Arch
Dermatologica 1989; 179: 57-8. Dermatol 1988; 124: 178 -9.
18. Boffa MJ, Chalmers RJ. Allopurinol- 23. Spencer JM, Silvers DN, Grossman
induced toxic pustuloderma. Br J ME. Pustular eruption after drug
Dermatol 1994; 131: 447. exposure: is it pustular psoriasis or a
19. Lotem M, Ingber A, Segal R et al. pustular drug eruption? Br J Dermatol
generalized pustular drug rash 1994; 130 : 514-19.
induced by hydroxychloroquine. Acta
163
News
National events International events

2004 2004
December 9-12, 2004 November 17-21

Silver Jubilee Conference of Pakistan 13th Congress of the European Academy
Association of Dermatologists, Karachi. of Dermatology and Venereology
Organizing Chairman: Dr. Khurshid H. Contact: Torello M. Lotti, Florence, Italy
Alvi, Suite No. 11, 3rd Floor, Taj Medical E-mail: president@eadv2004.org or
Complex, M.A. Jinnah Road, Karachi, info@eadv2004.org
74400 Pakistan
Tel: +92 21 7789666
Fax: +92 21 7789677 2005
E-mail: silver@pad.org.pk
info@pad.org.pk February 3-6
Website: www.pad.org.pk 4th South Asian Regional Conference of
Dermatology, Venereology & Leprology
DERMACON 2005
Inauguration of De partmental Website and New Delhi, India
Teledermatology Unit, Department of Contact: Dr. Vijay K. Garg
Dermatology (Unit I), King Edward Tel: 020 7383 0266
Medical College/Mayo Hospital, Lahore E-mail: info @dermacon2005.com
www.dermacon2005.com
On 25 th August, 2004, official website and
Teledermatology unit of Department of October 12-15
Dermatology (Unit I), Medical college/Mayo European Academy of Dermatology and
Venereology Congress (EADV)
Hospital, Lahore) were inaugurated. Initially,
London, UK
only store-and-forward type of teleconsultation Contact: Marilyn Benham
was started. Tel: 020 7383 0266
E-mail: eadv@bad.org.uk
www.eadv.org
164
Information for Authors
Manuscripts legends for illustrations. Each section should begin
The JPAD agrees to accept manuscripts prepared in on a new page. Generic names of the drugs should
accordance with the “Uniform Requirements for be used. Full names with abbreviations must be
Manuscript Submission to the Biomedical Journals” used given with the first mention, thereafter the
approved by the International Committee of abbreviation will be used. Abbreviations should be
Medical Journals Editors. Three copies of all used for unwieldy names or where the names occur
material for publication should be sent to Dr. Ijaz frequently. For all quantitative measurements the
Hussain, Editor, JPAD, Department of International System of Units (SI) should be used.
Dermatology, Mayo Hospital, Lahore, e- mail:
dr_ijazhussain@hotmail.com References
dr_ijazhussain@yahoo.com Only papers closely relevant to the author’s work
Manuscripts should be printed on one side of paper should be referred to. References should be in the
only, with a 2.5 cm margin on either side, be Vancouver style i.e. references should be written as
double spaced, and bear the title of the paper, name unbracketed superscript numbers in the order in
and address of each author, together with the name which they appear in the text e.g. ‘our previous
of the hospital, laboratory or institution where the reports1 and that of Cohen et al.2…..’. At the end of
work has been carried out. The name and full the article, references should give the name(s) and
address of corresponding author should be given on initials of author(s). If there are more than four
the first page. Pages should be numbered. Authors authors, include the first three authors followed by
should keep a copy of the manuscript. et al., title of paper, title of the journal abbreviated
in the standard manner (as published in the Index
In addition to the hard copy, an exact copy of the Medicus), year of publication, volume number, and
manuscript, containing all parts of the paper, must first and final numbers of the article, e.g. Grattan C,
be submitted on high-density disk. The editor Powell S, Humphreys F. Management and
reserves the right to make corrections, both literary diagnostic guidelines for urticaria and angio-
and technical, to the papers. Papers received are oedema. Br J Dermatol 2001; 144: 708-14.
supposed to have been submitted exclusively to the References to books should give the name(s)
Journal of Pakistan Association of Dermatologists followed by initials of author(s) or editor(s), chapter
and all authors must give a signed consent to (if relevant), book title, edition, place, publisher,
publication in a letter sent with the manuscript. year, and pages referred to e.g. Friedman WF, Child
Authorship implies a significant contribution. In JS. Congenital heart disease in the adult. In: Fauci
case of clinical trials, the names of pharmaceutical AS, Braunwald E, Isselbacher KJ et al., editors.
sponsors should be mentioned. Harrison’s principles of internal medicine. 14th edn.
New York: McGraw-Hill; 1998. p. 1300-9.
Types of articles
JPAD welcomes original and review articles, case Tables
reports, quizzes, items of correspondence etc. There should be as few tables as possible and these
addressing any aspect of dermatology. should include only essential data. These should be
printed on separate sheets and should be given
The original article should be of about 2000 words, Arabic numbers. No horizontal or vertical rules
with no more than 6 tables or illustrations. Letters should be used. Avoid wordy, over-full tables.
should not normally exceed 400 words and have Legends should be provided.
more than 10 references.
Illustrations
Parts of the paper Three sets of illustrations should be sent with each
The manuscript should be prepared as below. manuscript. Illustrations should be referred to in the
Title: In addition to the full title of the paper, a short text as ‘Figures’ and be given Arabic numbers. Each
version not more than 50 characters, for a running figure should be marked on the back with the name
head, be provided. of the author(s), the title of the paper and the
Author(s) details: Name(s) of the author(s) should reference number used in the text. Orientation of the
be given as initial(s) followed by surnames, and illustration should be indicated by marking the top
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by the use of symbols e.g. ∗ , †,‡ etc. prints. Diagrams should be on separate sheets and a
Abstract: All articles other than correspondence legend should be provided for each illustration.
should have an abstract. The original articles should
have a structured abstract comprising of 4 Proofs
subheadings: background, methods, results and Page proofs will be sent, without the original
conclusions. Keywords ≤ 5 should be provided to manuscript, to the corresponding author for proof
aid indexing. correction and should be returned to the editor
Main text: The main text should appear in the within three days. Major alterations from the text
following sequence: introduction, methods, results, cannot be accepted. Any alterations should be
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Journal of Pakistan Association of

Advisory Board Editorial Board

JPAD, the official journal of Pakistan Association of Dermatologists is published quarterly,

Cutaneous leishmaniasis in Sadda, Kurram Agency, Pakistan 114

Olive oil: an effective emollient for lichen simplex chronicus 118

Porokeratosis : A review of unique group of keratinizing disorders 130

Management of atopic dermatitis: a review 140

Information for authors 165

The most challenging task is the 5. Hollstein M, Sidransky D, Vogelstein

Abstract Background Systemic lupus erythematosus (SLE) is a multisystem disease of autoimmune

Key words SLE, neonatal LE, cutaneous manifestations.

Introduction Caucasian children with a male to female

Figure 2 Ulcers over lips and buccal mucosa.

Figure 1 Malar rash, photosensitive

Figure 4 Bilateral purpuric eruption on the

previous reports give a female

Table 4 Comparis on with study in children.

Patients and methods CL patients presenting to leishmaniasis clinic of Tehsil Headquarter

Introduction and Chakwal districts in the province of

belt bordering Afghanistan was the home of Leishmania in a Giemsa-stained sm ear

Kurram Agency is divided into three parts Results

Table 2 Anatomical distribution of lesions

Smear for Leishman-Donovan bodies is leishmaniasis in Multan. J Pak Med

Key words Olive oil, emollient, lichen simplex chronicus.

Introduction massaged and it was considered remedy of

Females Group A3 10% of the patients had

Group B1 30% patients had Females

Group A1 21-30 years n=10

Group A2 31-40 years n=10

Group A3 41-50 years n=10

Group B 10-20 years n=10

effects, so not really an ideal therapy. 10 3. Jossa F, Macinic M. The

Key words Guttate psoriasis, streptococcal pharyngitis, ASO titer

Introduction comprises immune-mediated cutaneous

Table 1 Demographic data of study population Discussion

Cases Controls This study provides the evidence that

Table 2 Clinical features of guttate psoriasis

study, 60% of patients with guttate management of acute guttate psoriasis.

Abstract Porokeratosis is a group of disorder of uncertain cause characterized by abnormal epidermal

Introduction seen at any age, from birth to adulthood, PM

Porokeratosis was first described by Mibelli21 in

Linear porokeratosis No definite inheritance anywhere, including the mucous membranes,

1. Porokeratosis of Mibelli [Figure 1] Non-actinic forms may be seen following

irregularly shaped, annular plaque with a raised,

Figure 4 Porokeratosis palmaris plantaris et

Figure 2 Disseminated superficial actinic

Figure 5 Porokeratosis palmaris plantaris et

association with other forms of porokeratosis.

5. Porokeratosis palmaris et plantaris have a high incidence of malignant

Multiple, asymptomatic, tiny, seed-like, 13. Malignant porokeratosis

disorders, such as cutaneous T cell lymphoma Course and Prognosis

cultured dermal fibroblasts. Cancer Genet porokeratosis like lesions. J Am Acad

Introduction to educate the patient and family in certain

vast majority of cases, improved bathing inhibition of leucocyte recruitment and

d. UVB therapy inflammatory dermatoses. Tacrolimus

radioallergosorbent tests (RASTs), who e. Naphthalan

applications significantly improve the

transplants. In doses of 2 or 3 g/d the drug special silk fabric (MICROAIR

b. Traditional Chinese medicine f. Hypnosis

h. Natural honey and beeswax 3. Jenner N, Campbell J, Marks R.

blind, parallel group study. BMJ eczematous dermatitis. Clin

J Allergy Clin Immunol 1997; 99: Japanese patients with intractable

Understanding the fundamentals of biostatistics is essential for every clinician in order