Original paper
Telangiectasia macularis eruptiva perstans as the first clinical presentation of systemic mastocytosis

Mastocytosis is a heterogeneous disease characterized by excess proliferation and accumulation of mast cells MC in one or more organs. Cutaneous mastocytosis (CM) is diagnosed on the basis of typical cutaneous lesions and an increase in infiltration of cutaneous MC after the elimination of systemic involvement. Telangiectasia macularis eruptiva perstans, currently named telangiectatic CM, is a rare subset of CM, considered to be a purely cutaneous form of mastocytosis. We present three cases of telangiectatic CM in which diagnostic procedures revealed systemic mastocytosis (SM) in two cases. In these patients skin lesions occurred as the first clinical presentation of SM; their histopathology examination revealed bone marrow involvement, serum tryptase levels were highly elevated, and bone marrow MC expressing CD2 and CD25 were found by flow cytometric analysis. According to WHO criteria in these two cases Indolent SM (ISM) was diagnosed; the third patient requires further clinical observation. Therapy included oral antihistamines (in all patients), PUVA (in one patient) and oral corticosteroids (in one patient) to reduce MC mediator related symptoms.