Coexistence of two types of clinical lesions in childhood-onset mastocytosis.

[cutaneous mastocytosis]

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 .