Mastocytosis with clinical features of histyocitosis.

Abstract

Cutaneous mastocytosis is a rare disorder characterized by an increased number of mastocytes in the dermis. Pseudoxanthomatous mastocytosis is a rare clinical variant reported by Fox in 1883, clinically characterized by yellowish and brownish papules and nodules, wich are reminiscent of xanthoma or histiocytosis. The Darier sign is slightly positive.