Telangiectasia macularis eruptiva perstans [TMEP] case report with review of the literature

ABSTRACT

The most frequently affected organ in any form of mastocytosis is the skin [1]. Cutaneous lesions tend to appear early in life and include urticaria pigmentosa, mastocytoma, diffuse and erythematous Cutaneous mastocytosis and Telangiectasia Macularis Eruptiva perstans [2, 3]. Telangiectasia Macularis Eruptiva Perstans is observed in less than 1 % of cases of mastocytosis [4]. Although cutaneous mastocytosis appears to occur sporadic, yet familial incidence was reported as four cases of telangiectasia macularis eruptiva perstans appearing in three generations of one family with an autosomal dominant mode of transmission with incomplete penetrance [5]. Telangiectasia macularis eruptiva perstans is characterized by telangiectatic reddish brown macules usually on the trunk, pruritus [6] with little tendency to urticate [7]. Telangiectasia macularis eruptiva perstans is mostly seen in adults and is occasionally seen in children or infants[8] The classic symptoms in Telangiectasia macularis eruptiva perstans include episodic flushing, gastrointestinal complaints, heart palpitation, syncope and may be confused with carcinoid [8]. We present a case of telangiectasia macularis eruptiva perstans in an adult and discuss its diagnosis and mastocytosis in general and its management