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ARTICLE IN PRESS

Current Paediatrics (2003) 13, 418 -- 422



c 2003 Published by Elsevier Ltd.
doi:10.1016/S0957-5839(03)00084- 8

Recognition and treatment of psoriasis in children


J.A. Leman and A.D. Burden

Department of Dermatology, Royal Hospital for Sick Children, Glasgow, G3 8SJ, UK

KEYWORDS Summary Psoriasis frequently presents in childhood and is usually easily recognized.
childhood psoriasis; genetic Mild disease or an atypicalpresentation maypresentdiagnostic diff|culty.There are many
factors; environmental clinical patterns, and anogenital, facial and guttate lesions are particularly well recog-
triggers; clinical forms; nized in this age group. Education of children and parents about disease chronicity and
management strategies treatment options is essential for successful management. Most patients respond to to-
pical treatment with emollients, coal tar, dithranol or calcipotriol. Supervision of the ap-
plication of topical medicaments in a daycare setting, or admission to a ward, may be
necessary if response to outpatienttreatment is disappointing.Phototherapy with ultra-
violet B may be combined with topical treatment in older children with more extensive
disease. A minorityof childrenwith particularly severe disease require systemic therapy.
Retinoids are the most commonly used agents.There are few published data regarding
methotrexate or cyclosporin in childhood psoriasis, although these agents appear to
have a role in those most severely affected.

c 2003 Published by Elsevier Ltd.

tion with lifetime risks of 4%, 28% and 65% if neither, one
PRACTICE POINTS or both parents, respectively, are affected.1 Nine named
genetic susceptibility loci (PSORS1--PSORS9) have been
* Childhood psoriasis can usually be suppressed ade- detected by linkage analysis, but the genetic determi-
quately with topical agents nants at these loci have not yet been identif|ed. The sus-
* Adequate disease control requires a high degree of ceptibility locus with the greatest genetic effect
parental motivation (PSORS1) lies on chromosome 6p21.3 in the region of
* In severe psoriasis, phototherapy or systemic ther- the major histocompatability complex.2 Psoriasis has
apy can be highly effective been subdivided by age of onset.3 Type 1 psoriasis has an
age of onset o40 years, is often familial and is strongly
associated with HLA Cw6. In contrast, presentation la-
ter in life (type 2 psoriasis) is less often familial and is
INTRODUCTION not associated with the Cw6 allele.
Psoriasis is an inherited, chronic, inflammatory condition
of the skin that affects about 2% of the population and
often presents in childhood.The clinical course is unpre- CLINICAL FEATURES
dictable, characterized by remissions and relapses. On
histological examination, plaques reveal hyperprolifera- Psoriasis is diagnosed prior to the age of 2 years in 2% of
tion and abnormal differentiation of keratinocytes, vas- cases and before10 years in10% of cases.The age at onset
cular endothelial proliferation and inflammatory cell is younger in girls than boys. Establishing the diagnosis in
inf|ltration. The aetiology and pathogenesis of psoriasis infancy can be challenging because of limited involve-
remain enigmatic. It is likely that there is interplay be- ment or an atypical appearance. Under these circum-
tween genetic and environmental factors. T cells play a stances, observing the natural history as it evolves is
pivotal role, and the cytokine pattern is skewed towards often more helpful than taking a skin biopsy in reaching
Th1.There is compelling evidence of a genetic predisposi- a diagnosis.
All of the clinical variants of psoriasis described in
Correspondence to: JAL.Tel.: +44(0)141211 6259; adults are recognized in childhood.4 Different forms
E-mail: Joyce.leman.wg@northglasgow.scot.nhs.uk. may occur in the same patient at different times. Plaque
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RECOGNITION ANDTREATMENTOF PSORIASIS 419

psoriasis is the most common variant encountered in


children.Typical lesions are 5--10 cm in diameter, symme-
trically located over extensor surfaces of the limbs.Facial
involvement appears to be particularly common in chil-
dren compared with adults (Fig.1).The scalp is frequently
involved, and this must be distinguished from sebor-
rhoeic dermatitis or tinea capitis.
The involvement of flexural surfaces is particularly
common in young children.The napkin area is frequently
the f|rst site affected, with well-def|ned erythema devoid
of scale. At this site, psoriasis must be differentiated
from irritant contact dermatitis and seborrhoeic derma-
titis. Napkin seborrhoeic dermatitis is a particular diag- Figure 2 Guttate psoriasis is characterized by the eruption of
nostic problem as a signif|cant proportion of affected small papules of psoriasis, often related to a streptococcal sore
children develop psoriasis in adulthood.5 Psoriatic nail throat.
changes (often misdiagnosed as fungal infection) are
characterized by superf|cial pits, onycholysis and subun- psoriasis over the next few years. Pityriasis rosea and
gal hyperkeratosis. discoid eczema may cause diagnostic confusion.
Guttate psoriasis develops suddenly, often in response Erythrodermic and pustular variants of psoriasis are
to a streptococcal infection of the throat or skin. It is rare in childhood. Erythrodermic psoriasis may be preci-
characterized by the eruption of multiple papules 0.5-- pitated by drugs such as antimalarials. Pustular psoriasis,
1cm in diameter over the face, trunk and limbs (Fig. 2). a potentially life-threatening disorder in adults, often fol-
Infection may be conf|rmed by culturing a throat swab lows a more benign course in childhood. It may be preci-
and by measuring the serum antistreptolysin O titre. pitated by infection, corticosteroids or ultraviolet B
Although most clinicians will treat any associated infec- exposure.The pustules, which are superf|cial and sterile,
tion, evidence that this will ameliorate the psoriasis is may be generalized or localized.General malaise, pyrexia
lacking.6 Lesions typically persist for a few months before and anorexia are frequent accompaniments. Pustular
resolving spontaneously. However, many patients psoriasis may be the f|rst presentation of psoriasis. In
experience a recurrence of either guttate or plaque about one-third of cases, there is a preceding history of
seborrhoeic dermatitis or napkin dermatitis.
Psoriatic arthropathy, a seronegative inflammatory ar-
thritis, is uncommon in childhood. The long-term prog-
nosis is usually favourable. Distinction should be made
from juvenile rheumatoid arthritis and ankylosing spon-
dylitis.7

TREATMENT
For the majority of patients, psoriasis is mild and easy to
control. In a minority, the disease presents a challenging
management problem. Customising treatment to reflect
patient age, extent, severity and location is imperative. In
younger patients, compliance often reflects parental mo-
tivation. From the outset, it should be emphasized that a
realistic objective is to control rather than cure the dis-
ease. The possibility that factors such as streptococcal
infection, psychological stress or trauma (the Koebner
or isomorphic phenomenon) could be contributing to
ongoing activity or flares should be considered.

Topical treatment
Most childhood psoriasis will respond well to topical
Figure 1 Involvement of the face by psoriasis is a particularly agents. Cosmetic acceptability of agents should be con-
common problem in children. sidered as this can greatly influence patient compliance.
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420 CURRENT PAEDIATRICS

Most patients can apply topical treatments at home un- flexural, face and genital lesions, and burns healthy peri-
der parental supervision. Initial demonstration of appli- lesional skin. Parental supervision of application and the
cation of treatments by skilled dermatology nurses may use of a protective greasy emollient around plaques are
be helpful. If response to therapy is poor or disease is advocated.
particularly extensive, attendance at a day treatment
centre or admission to an inpatient facility may be helpful Vitamin D analogues
if available. Vitamin D stimulates differentiation and inhibits DNA
synthesis and cell proliferation in cultured keratinocytes.
Emollients In addition, a role in immunomodulation is postulated.
The application of an emollient reduces scaling and re- However, effects on calcium homeostasis restrict the
lieves itch. They are inexpensive and well tolerated. In clinical use of vitamin D3.Calcipotriol, a synthetic analo-
mild disease, this may be the only therapy required. gue of vitamin D3, has a lesser effect on calcium home-
ostasis. Patient acceptability is high as the preparation is
Topical corticosteroids clean and odour free. In a UK multicentre open study of
Topical corticosteroids are particularly useful when itch 66 children, calcipotriol 50 mg/g was applied twice daily
is a major symptom. Their lack of odour and ease of ap- for up to 8 weeks (maximum 45 g/week/m2). Clinical
plication make them popular with patients. They have clearance or marked improvement was documented in
anti-inflammatory and antiproliferative effects, reducing 65% of cases, and the psoriasis area and severity index
erythema and scaling of plaques. They may be used as (PASI, a standard measure of disease extent) was signif|-
monotherapy on the flexures, face or scalp-sites prone cantly reduced.9 An international, double-blind, parallel
to irritation from other topical preparations. On stable group study in 77 children compared calcipotriol 50 mg/
plaque disease, they are often combined with other g twice daily with placebo over an 8 -week period.10 Tol-
agents such as calcipotriol. In general, the least potent erability of the treatment was conf|rmed but the fall in
topical corticosteroid should be used for the shortest PASI was not signif|cantly different in the two groups.
possible time to minimize the risks of long-term sequelae Calcipotriol may cause irritation if applied to the face,
such as striae and telangiectases. Application under oc- napkin or genital areas. In the UK, calcipotriol 50 mg/g is
clusion may be clinically benef|cial but does lead to en- licensed for use in children at a maximum dose of 50 g/
hanced penetration and therefore potentiation of week in those aged over 6 years and 75 g/week in those
adverse effects. Extensive application can lead to sup- aged over 12 years. Tacalcitol and calcitriol are also
pression of the hypothalamic--pituitary--adrenal axis. Re- vitamin D analogues that are commercially available,
bound flare of disease is well recognized if topical but there are no published data concerning their use in
corticosteroids are withdrawn abruptly. A gradual children.
reduction is advocated.
Topical retinoids
Tars Tazarotene is a receptor-specif|c topical retinoid licensed
Tars are widely used in the treatment of psoriasis, but for use in adults. However, it remains untested in child-
their odour and tendency to stain limit their acceptabil- hood psoriasis. In adults, local irritation is commonly
ity to patients.They have antiproliferative and antipruri- reported and may be ameliorated by the additional use
tic effects. Irritancy is low and tars such as coal tar or of a topical steroid.
ichthyol may be prescribed for sensitive sites such as the
face and flexures, as well as plaques on trunk and limbs. Topical immunomodulators
Combining tar and ultraviolet B (Goeckerman regimen) Tacrolimus inhibits T-cell activation and has been shown
may be helpful, especially for extensive plaque or guttate to be effective when administered orally in adults with
disease. chronic plaque psoriasis. In pilot studies, non-occluded
topical application is no more effective than placebo. This
Dithranol (Anthralin) may reflect poor penetration into thick psoriatic pla-
Dithranol is an effective treatment, particularly for ques. In childhood, facial psoriasis is common and the
large, thick plaques. In a study of children aged 5--10 stratum corneum at this site is thin. Topical tacrolimus
years, remission was achieved using dithranol in 47 of 58 0.1%, applied twice daily for 4 weeks to facial lesions, has
(81%) children.8 The application of dithranol (0.1% up to been reported to be effective in 10 of 11 adult patients.11
3%) washed off after 30 -- 45 min maintains eff|cacy but
reduces side effects. A cream base preparation is avail-
Phototherapy
able for home use. Daycare patients and inpatients are
often treated with dithranol in Lassars paste which is Ultraviolet B (UVB) phototherapy is highly effective in
more effective but less cosmetically acceptable. Dithra- inducing a remission in widespread psoriasis. The dura-
nol stains skin and clothing, has a propensity to irritate tion of remission is variable between patients but can last
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RECOGNITION ANDTREATMENTOF PSORIASIS 421

for months or even years. Increasingly, fluorescent tubes Non-invasive techniques such as serial measurement of
that emit a wavelength of 31172 nm (narrow-band UVB) serum procollagen III peptide are under investigation.
are used as this appears to be more effective than pre-
viously used broad-band UVB. Treatment is usually given
three times a week for about 6 weeks with increasing Cyclosporin
doses as tolerated. Combining UVB and topical prepara- Cyclosporin is a potent inhibitor of IL-2 production from
tions such as tar or calcipotriol is benef|cial. Side effects T lymphocytes. Literature on its use in childhood psoria-
of UVB include immediate burning and delayed photocar- sis is sparse but there is considerable experience in child-
cinogenicity. Particular concerns about increased photo- hood atopic eczema and in adult psoriasis. Cyclosporin is
carcinogenesis in children limit the use of UVB in this age best used to achieve remission rather than for mainte-
group, as do the practical diff|culties of administering the nance therapy.
treatment in young children.12 A single case was reported of a 9-year-old boy with
generalized pustular psoriasis who responded to cyclos-
porin 3 mg/kg/day.17 However, four patients with eythro-
Systemic therapy dermic or pustular psoriasis failed to respond adequately
In adults with psoriasis, retinoids, cyclosporin and meth- to cyclosporin (2.5--7.5 mg/kg/day).18 In one of these four
otrexate are the most frequently used agents but data patients, cyclosporin was accidentally given at 10 mg/kg/
relating to their use in childhood psoriasis are lacking. day and was effective at this dose.The use of cyclosporin
Only in the most severe cases of psoriasis (pustular, ery- in the paediatric transplant population suggests that
throdermic, arthropathic and extensive resistant plaque children may require a relatively higher dose or a more
psoriasis) are such agents appropriate. Their introduc- frequent dosing regimen than adults. 19
tion should be made under the supervision of an experi-
enced dermatologist, and parents must be advised of
possible adverse effects and the need for monitoring.
CONCLUSIONS
Parental and physician attitudes can have a major impact
Retinoids
on the disability caused by psoriasis. The disease can
Acitretin (0.25-- 0.6 mg/kg) is the most commonly used
usually be well controlled with the use of topical agents,
agent. Only case reports and small case series support
but modern treatment remains suppressive rather than
its use in childhood psoriasis,13,14 although retinoids have
curative or disease-modifying.Only the most severely af-
been used extensively in inherited disorders of keratini-
fected are likely to require second-line agents, which
zation. Measurements of baseline lipid prof|le and liver
should be introduced only by experienced dermatolo-
enzymes are required, repeated at 3-monthly intervals.
gists and require monitoring for the duration of treat-
Girls of childbearing age must be counselled of the abso-
ment. The dramatic response of psoriasis to anti-TNFa
lute necessity of avoiding pregnancy during and for
therapy raises the possibility of newer, more effective
2 years after cessation of the drug. Dryness of the
treatments.20 The development of more selective immu-
skin and mucous membranes and myalgia are common
nomodulatory drugs for psoriasis is an area of very ac-
adverse effects.
tive research and development.

Methotrexate
Methotrexate has a direct effect on T lymphocytes as
well as on the keratinocyte cell cycle.There are few pub- REFERENCES
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