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获得性大疱性表皮松解症

Authors
David T Woodley, MD
Mei Chen, PhD
Gene Kim, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD
Translators
林麟, 主任医师

引言

获得性大疱性表皮松解症(epidermolysis bullosa acquisita, EBA)是一种罕见的、散发的、上皮下的、皮肤黏膜的疱性疾病，通常发生于成年期。EBA通常被描述为一种以皮肤易脆、非炎性紧张性大疱、粟粒疹和瘢痕形成为特征的机械性大疱病。或者，EBA可表现为炎性大疱疹，类似于大疱性类天疱疮(bullous pemphigoid, BP)或其他上皮下的自身免疫性大疱病。

EBA的发病机制涉及抗Ⅶ型胶原的抗体的产生，而Ⅶ型胶原是皮肤和黏膜基底膜带的锚原纤维的一种主要组成成分(图 1)。免疫介导的锚原纤维的破坏很可能促成了基底膜带分裂和临床水疱的形成。

EBA的发病机制、临床表现和治疗将在此讨论。遗传性大疱性表皮松解症见其他专题。 (参见“大疱性表皮松解症的流行病学、发病机制和临床表现”和“大疱性表皮松解症的诊断”和“大疱性表皮松解症治疗概述”)

术语

虽然“EBA”的名称源于最初认为它是一种类似于隐性营养不良性大疱性表皮松解症(recessive dystrophic epidermolysis bullosa, RDEB)的疾病，但EBA和大疱性表皮松解是不同的疾病。RDEB是一种遗传性疾病，由编码Ⅶ型胶原的COL7A1基因突变引起的。与EBA不同，RDEB通常在婴儿期首次发病。 (参见“大疱性表皮松解症的诊断”)

流行病学

EBA是一种发病率不明确的罕见疾病。虽然已有儿童病例的报道，但成人最常受累[1-4]。尚不明确EBA的风险是否存在基于性别、种族或地理位置的增加。

                         

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Literature review current through: 2017-06 . | This topic last updated: 2015-06-30.
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