Cohn M S, Mahon M J
Department of Dermatology, Pontiac Osteopathic Hospital, Mich.
J Am Osteopath Assoc. 1994 Mar;94(3):246-8.
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.
持久性发疹性斑状毛细血管扩张症（TMEP）是肥大细胞增多症的一种罕见形式。它最常出现在成年人中，偶尔也会影响幼儿或婴儿。在这种疾病中，主要在躯干上会出现多个棕红色融合性斑疹和毛细血管扩张。瘙痒经常发生，程度可轻可重。大多数患者仅累及皮肤；然而，也可能累及全身。系统性肥大细胞增多症的临床体征和症状各不相同，取决于受影响的内部器官。典型症状——如发作性潮红、胃肠道不适、心悸和晕厥——可能与其他疾病的症状相混淆，最显著的是类癌综合征。一项简单的检查有助于区分这两种情况。作者描述了一名48岁女性，她有TMEP的皮肤特征，并伴有多种提示系统性肥大细胞增多症的症状。他们讨论了这种相对罕见疾病的临床特征、诊断检查和治疗选择。
