Telangiectasia macularis eruptiva perstans (TMEP) is a rare form of mastocytosis. It appears most frequently in adults and only occasionally will affect young children or infants. In this disease, multiple brownish-red confluent macules and telangiectasias develop, primarily on the trunk. Pruritus frequently occurs, and may be mild to severe. Most patients have only skin involvement; however, involvement may be systemic as well. Clinical signs and symptoms of systemic mastocytosis are varied and depend on which internal organs are affected. Classic symptoms--such as episodic flushing, gastrointestinal complaints, heart palpitations, and syncope--may be confused with those of other diseases, most notably the carcinoid syndrome. A simple workup can help to differentiate between these two conditions. The authors describe a 48-year old woman who was seen with cutaneous features of TMEP and with multiple symptoms suggesting systemic mastocytosis. They discuss the clinical features, diagnostic workup, and therapeutic options in the management of this relatively rare condition.

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