606 LETTERS TO THE EDITOR

Treatment with efalizumab was stopped, and Goeckerman cure 6 Santos-Juanes J, Coto-Segura P, Mallo S, Galache C, Soto J. Multiple
was started. Application of Gentamicin and hidrocortisona 1% eruptive dermatofibromas in a patient receiving efalizumab. Dermatology 2008;
216: 363.
cream in the axillae, perineum and submammary area twice a day
was begun. After 18 sessions, the psoriasis improved (PASI 4) and DOI: 10.1111/j.1468-3083.2008.02979.x
the lesions of FBCP remained stable. Letters to the
XXX
LETTERS
LETTERS TO
TOEditor
THE
THEEDITOR
EDITOR

Various adverse cutaneous reactions have been reported during

anti-CD11a treatment such as the appearance of multiple erup-
tive dermatofibromas.6 Efalizumab (anti-Cd11a) is a humanized
monoclonal antibody, which blocks the multiple T-cell–dependent
Di-isostearyl malate and
functions involved in the pathogenesis of psoriasis, including macademia nut oil in lipstick
T-cell activation and migration to the skin, provoking a partial
immunosuppressed state.
caused cheilitis
Although we cannot exclude it, we believe that this association
Editor
and the role of efalizumab are not just coincidental. Psoriasis is
Di-isostearyl malate and macademia nut oil are known ingredients
relatively common, but FBCP is less common. The simultaneous
of cosmetic agents. Di-isostearyl malate is composed of isostearyl
occurrence of these two disorders may indicate that the genes
alcohol and malaic acid. Isostearyl alcohol has branched C18 aliphatic
responsible for them are closely linked or associated.2 In these
compounds, and this agent caused lipstick dermatitis.1 Teuber2
patients, genetically predisposed, certain stimulating factors could
reported that tree nut and peanut oil may be a threat for patients with
led to the development of FBCP.8 The underlying mechanisms of
allergies depending on the method of manufacturing and processing.
the development of the FBCP lesions remain unclear. It is possible
A 28-year-old woman had presented cheilitis after using a lipstick
that the efalizumab could have triggered or facilitated the
(Fig. 1). The patient had no history of any drug or cosmetic
manifestation of pemphigus and simultaneously provoked the
allergies. We performed patch tests using the ingredients of the
worsening of psoriasis. The patient had never suffered lesions on
lipstick; checking for contact dermatitis. The patient had positive
the axilla, groin or submammary region. Bi-directional isomorphism
reactions to di-isostearyl malate and macademia nut oil (Fig. 2).
could explain the psoriatic plaques and also the FBCP plaques on
Because the agents of highest concentration in the lipstick were
the folds.3
di-isostearyl malate and macademia nut oil, it was recommended
We choose phototherapy in this case, because the patient
that the patient use another lipstick without these agents. The
reported that NB-UVB and PUVA have helped his psoriatic con-
patient improved by using a steroid ointment.
dition. Furthermore, improvement of FBCP has been previously
There were some reports about contact dermatitis on the lips.3–5
reported in the literature.1,4 In our patient, Goeckerman cure led
Previously, Jere6 and Sugiura7 reported cheilitis due to di-isostearyl
to improvement of the psoriatic plaques, but FBCP plaques
malate in the lipstick. This was a rare case of allergic contact
remained more or less stable.
cheilitis due to both di-isostearyl malate and macademia nut oil.
Generally, di-isostearyl malate is commonly used in lipstick and
J Santos-Juanes,†* P Coto-Segura,† J Saavedra,‡ in about 10% of lip glosses. Hayakawa1 reported that di-isostearyl
S Laviano,‡ C Galache† alcohol in the di-isostearyl malate contained C18 aliphatic
†
Servicio de Dermatología II, Hospital Universitario Central de Asturias,
compounds and caused cheilitis. Macademia nut oil is a vegetable
Universidad de Oviedo, Oviedo, Spain, ‡Servicio de Dermatología, Hospital
oil from macademia nuts and includes oleic acid and squalane.
Valle del Nalón, Oviedo, Spain
*Correspondence: J Santos-Juanes. E-mail: jsantosj@hca.es

References
1 Heaphy MR, Winkelmann RK. Coexistence of benign familial pemphigus and
psoriasis vulgaris. Arch Dermatol 1976; 112: 1571–1574.
2 Fischer I, Orkin M, Bean S. Familial benign chronic pemphigus and
psoriasis vulgaris in the same patient. Acta Derma Venereol 1967; 47:
111–117.
3 Boxley JD, Byrne JPH, Summerly R. Bi-directional isomorphism: coexistence
of psoriasis vulgaris and Familial Benign Chronic Pemphigus. Arch Dermatol
1977; 113: 846–847.
4 Hayakawa K, Shiohara T. Coexistence of psoriasis and familial benign chronic
pemphigus: efficacy of ultraviolet B treatment. Br J Dermatol 1999; 140:
358–377.
5 Mallen JK. Psoriasis, chronic benign familial pemphigus, and
dysplastic naevus syndrome in a family. Australas J Dermatol 1992; Figure 1 Clinical appearance of cheilitis.
33: 55.

© 2008 The Authors

JEADV 2009, 23, 570–620 Journal compilation © 2008 European Academy of Dermatology and Venereology
LETTERS TO THE EDITOR
Figure 2 Positive reactions.
This oil is often used for lipstick, conditioner and other cosmetic
products. Cheilitis from macademia nut oil has never been
reported to our knowledge.
K Sugiura,* M Sugiura
Department of Environmental Dermatology and Allergology, Daiichi Clinic,
Nagoya, Japan
*Correspondence: K Sugiura. E-mail: ksugiura@daiichiclinic.jp
References
1 Hayakawa R, Matsunaga K, Suzuki K, Suzuki M, Arima Y, Ohkido Y. Lipstick
dermatitis due to C18 aliphatic compounds. Contact Dermatitis 1987; 16:
215–219.
2 Teuber SS, Brown RL, Haapanen LA. Allergenicity of gourmet nut oils
processed by different methods. J Allergy Clin Immunol 1997; 99: 502–507.
3 Tan BB, Noble AL, Roberts ME, Lear JT, English JSC. Allergic contact
dermatitis from oleyl alcohol in lipstick cross-reacting with ricinoleic acid in
castor oil and lanolin. Contact Dermatitis 1997; 37: 41–42.
4 Natalia P, Juan Francisco S, Javier M, Ana L, Maria P. Contact cheilitis from
bisabolol and polyvinylpyrrolidone/ hexadecene copolymer in lipstick.
Contact Dermatitis 2008; 58: 178–179.
5 Serra-baldrich E, Puig LL, Gimenez A, Camarasa JG. Lipstick allergic contact
dermatitis from gallates. Contact Dermatitis 1995; 32: 359–372.
6 Guin Jere D. Allergic contact cheilitis from di-isostearyl malate in lipstick.
Contact Dermatitis 2001; 44: 375.
7 Sugiura M, Hayakawa R, Kato Y, Sugiura K, Hashimoto R, Shamoto M. Three
cases of lip dermatitis due to diisostearyl malate. Environ Dermatol 2001; 8:
6–10.
DOI: 10.1111/j.1468-3083.2008.02983.x
JEADV 2009, 23, 570–620
607
An atypical form of dyskeratosis
Letters to the
XXX
LETTERS
LETTERS TO
TOEditor
THE
THEEDITOR
EDITOR
congenita with renal agenesis
and no mutation in DKC1, TERC
and TERT genes
Editor
Dyskeratosis congenita (DC) is a rarely observed form of
ectodermal dysplasia characterized by dystrophic nails, reticulate
skin pigmentation, mucosal leukoplakia, aplastic anaemia and an
increased risk of malignancy.1 Mutations in DKC1 gene are
responsible for X-linked disease.2 The TERC gene is associated
with the autosomal-dominant form of the disease.3
A 19-year-old male patient was seen at the genetic department
because of nail dysplasia, short stature and pallor skin. His parents
were first cousins. Our patient was operated for undescended
testes at 13 years old. His weight was 39 kg (< 3rd centile); height
was 158 cm (< 3rd centile). Physical examination showed pale
appearance, defective and irregular teeth, dystrophic fingernails,
atrophic and shiny tongue. Oral leukoplakia was observed from
time to time. Secondary sex characters were delayed. He had a
bizarre pigmentation on his left leg which appeared 2 years ago
(Fig. 1). Punch biopsy from this lesion revealed hyperkeratinization
and basal pigmentation in the epidermis, and an inflammatory
infiltrate composed of mononuclear cells in the dermis, but
dermal pigmentation could not be demonstrated. Intravenous
pyelogram and renal scintigraphy demonstrated normal left
kidney function but no function of the right kidney. Hemoglobin
level was 8.9 mg/dL. White blood count was 4900/mm3. Thrombocyte
count was 216 000/mm3. Bone marrow aspiration revealed
hypocellular bone marrow with megaloblastic changes.
The patient was invited to our clinic for re-evaluation of his
disease (DC) at 26 years old. His weight was 50 kg (< 3rd centile);
height was 171 cm (10–25 centile) A combination of screening
by denaturing high-performance liquid chromatography
analysis and direct DNA sequencing found no abnormality in
the three most commonly mutated exons of the DKC1 gene as well
as the coding and flanking sequences of the TERC and TERT
genes.
DC is an inherited disease characterized by the triad of
abnormal skin pigmentation, nail dystrophy, mucosal leukoplakia.
Index case has one or more of these mucocutaneous features,
combined with a hypoplastic bone marrow and at least two of the
other somatic features known to occur in DC.4 These other
features include bone marrow failure, developmental delay, dental
caries, short stature, hypogonadism and osteoporosis.1 Our
patient had skin, nail and teeth abnormalities typical of DC. He
also had cryptorchidism, hypogonadism, short stature, growth
retardation which are diagnostic criteria of DC.
No abnormality has been detected in DKC1, TERC and TERT
genes in this subject. There is a small possibility that a mutation
© 2008 The Authors
Journal compilation © 2008 European Academy of Dermatology and Venereology