反向性营养不良型大疱性表皮松解症：一例报告

Dystrophic epidermolysis bullosa inversa: a case report.

作者信息

Altomare G F, Polenghi M, Pigatto P D, Nazzaro V, Piattoni F

机构信息

2nd Department of Dermatology, University of Milan, Italy.

出版信息

Dermatologica. 1990;181(2):145-8. doi: 10.1159/000247905.

DOI:10.1159/000247905

PMID:2173667

Abstract

The inverse form of recessive dystrophic epidermolysis bullosa is a rare genodermatosis characterized by a smouldering course of integumental blistering with improvement of lesions in adulthood, preferential localizations of lesions in flexural areas, severe oral and esophageal mucosal involvement and nail dystrophy. We describe a 41-year-old patient showing all the typical features of this form of epidermolysis bullosa. Ultrastructural findings in specimens obtained from perilesional and healthy skin were similar to those usually observed in the Hallopeau-Siemens form of epidermolysis bullosa. The patient has been treated with phenytoin for a period of 9 months with considerable improvement of the skin manifestations.

摘要

隐性营养不良性大疱性表皮松解症的反向型是一种罕见的遗传性皮肤病，其特征为皮肤水疱病程隐匿，成年期病变有所改善，病变好发于屈侧部位，口腔和食管黏膜严重受累以及指甲营养不良。我们描述了一名41岁的患者，其表现出这种大疱性表皮松解症的所有典型特征。从病损周围和健康皮肤获取的标本的超微结构 findings 与通常在Hallopeau-Siemens型大疱性表皮松解症中观察到的相似。该患者接受苯妥英治疗9个月，皮肤表现有显著改善。