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结节性硬化症中的生殖器血管纤维瘤:两例报告。

Genital angiofibromas in tuberous sclerosis: two cases.

作者信息

Nico M M, Ito L M, Valente N Y

机构信息

Department of Dermatology, University of São Paulo, Brazil.

出版信息

J Dermatol. 1999 Feb;26(2):111-4. doi: 10.1111/j.1346-8138.1999.tb03520.x.

Abstract

Cutaneous lesions are an important feature of tuberous sclerosis (TS). Facial angiofibromas usually begin to appear at the end of the first decade of life and are considered to occur in about 88% of adults with TS. They are only rarely reported on sites other than the face or front. In this paper, we report two patients with the complete syndrome of TS that had, in addition to classic facial lesions, multiple papules on the genital area. Histopathology confirmed the diagnosis of angiofibroma. One patient's lesions were misdiagnosed as genital warts and were so being treated before the correct diagnosis was made. Although we don't know the incidence of genital angiofibromas in TS patients, we believe that they might be underreported, because dermatologists generally don't look for them when they see these patients.

摘要

皮肤病变是结节性硬化症(TS)的一个重要特征。面部血管纤维瘤通常在生命的第一个十年末期开始出现,据认为约88%的成年TS患者会出现。它们很少出现在面部或前部以外的部位。在本文中,我们报告了两名患有完整TS综合征的患者,除了典型的面部病变外,生殖器区域还有多个丘疹。组织病理学证实为血管纤维瘤。一名患者的病变被误诊为尖锐湿疣,并在做出正确诊断之前一直如此治疗。虽然我们不知道TS患者中生殖器血管纤维瘤的发病率,但我们认为它们可能未被充分报告,因为皮肤科医生在诊治这些患者时通常不会特意寻找它们。

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