Boeira Vanessa Lys Simas Yamakawa, Souza Erica Sales, Rocha Bruno de Oliveira, Oliveira Pedro Dantas, Oliveira Maria de Fátima Santos Paim de, Rêgo Vitória Regina Pedreira de Almeida, Follador Ivonise
Federal University of Bahia (UFBA), Salvador, BA, Brazil.
An Bras Dermatol. 2013 Mar-Apr;88(2):185-98. doi: 10.1590/S0365-05962013000200001.
Inherited epidermolysis bullosa (EB) is a heterogeneous group of genetic disorders that present with skin and, in some cases, mucosal fragility, predisposing patients to the development of blisters and/or erosions after minimal trauma or friction. Children with a recurrent history of these kinds of lesions or neonates that present them in the absence of another reasonable explanation should be investigated. Diagnosis must be based on clinical and histopathological findings. To date, management of inherited EB basically consists in avoiding traumas that trigger lesions, as well as preventing infection and facilitating healing of the wounds with the systematic use of bandages.
遗传性大疱性表皮松解症（EB）是一组异质性的遗传性疾病，其表现为皮肤病变，在某些情况下还伴有黏膜脆弱，使患者在受到轻微创伤或摩擦后易出现水疱和/或糜烂。有这类病变复发史的儿童或无其他合理解释而出现此类病变的新生儿均应接受检查。诊断必须基于临床和组织病理学检查结果。迄今为止，遗传性EB的治疗主要包括避免引发病变的创伤，预防感染，并通过系统使用绷带促进伤口愈合。
