Epidermolysis bullosa: when the skin falls apart.
Epidermolysis bullosa: when the skin falls apart.
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大疱性表皮松解症：当皮肤破裂时。
DOI：
10.1016/s0022-3476(89)80550-5
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发表时间：
1989
期刊：
影响因子：
--
通讯作者：
Carter,DM
中科院分区：
文献类型：
--
作者：
Lin,AN;Carter,DM
如今，大疱性表皮松解症不再像几十年前那样是一个神秘的疾病。近年来，我们开始了解水泡形成背后的生化和结构缺陷。随着对临床和微观特征的了解不断增加，已识别出 17 种 EB 亚型，从而提高了制定预后的准确性。产前诊断已成功。尽管之前已经对 EB 进行了综述，但 1 个新的观察结果增强了我们对这种情况的理解。例如，新观察到的临床特征导致了新亚型的识别。据报道，新型外用抗生素（莫匹罗星软膏）取得了良好的治疗效果，并且培养表皮移植也取得了成功。在此，我们回顾并更新了 EB 的基础和临床方面。
Today, epidermolysis bullosa is no longer the mystery disease it was decades ago. In recent years, we have begun to understand the biochemieal and structural defects underlying blister formation. Increased knowledge about clinical and microscopic features has led to identification of 17 subtypes of EB, allowing improved accuracy in formulating prognosis. Prenatal diagnosis has been successful. Although EB has been reviewed previously, 1 new observations have enhanced our understanding of this condition. For example, newly observed clinical features have led to identification of new subtypes. Favorable treatment results with a new topical antibiotic (mupirocin ointment) have been reported, and success with cultured epidermal grafts has been achieved. Herein, we review and update basic and clinical aspects of EB.