Coexistence of two types of clinical lesions in childhood-onset mastocytosis
Date
2011-10-06
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Medknow Publications on behalf of The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL)
Abstract
Description
The vast majority of mastocytosis appear in childhood, urticaria
pigmentosa (UP) and mastocytomas being the most common types. Terms
such as "xanthelasmoid mastocytosis", "pseudoxanthomatous mastocytosis"
or "nodular mastocytosis" have been introduced in the literature to
describe the presence of yellowish papular or nodular lesions. We
describe two children with cutaneous mastocytosis showing yellowish
lesions in combination with other skin lesions. A 10-year-old girl
presented with asymptomatic lesions in her vulva at birth, and
developed brownish macules on her trunk years after. An eight- year-old
boy presented with multiple yellowish papular lesions on his trunk,
neck and limbs coexisting with a few clinically anetodermic lesions. No
systemic involvement was found and the skin biopsy confirmed a
cutaneous mastocytosis in both cases. The two patients are currently
asymptomatic and are being periodically followed up. Mastocytoses may
show a variety of clinical lesions, sometimes leading to misdiagnosis.
Although there are previous reports, involvement of the mucosae and
secondary anetoderma are not common findings in cutaneous mastocytoses.
We consider that cutaneous manifestations of mastocytoses compose a
clinical spectrum, thus explaining the coexistence of different
clinical lesions and the development of uncommon presentations.
Keywords
anetoderma, xanthelasmoid
mastocytosis, pseudoxanthomatous mastocytosis, Nodular mastocytosis
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