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获得性大疱性表皮松解症

Epidermolysis bullosa acquisita.

作者信息

Cobos Gabriela, Mu Euphemia, Cohen Jeffrey, Beasley Jenna, Brinster Nooshin, Femia Alisa

机构信息

New York University, New York.

出版信息

Dermatol Online J. 2017 Dec 15;23(12):13030/qt4jk4333w.

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare, acquired subepidermal blistering disease. EBA is characterized by autoantibodies to collagen VII,which serves to link the epidermis to the dermis. The two most common presentations of EBA are classical noninflammatory EBA and bullous pemphigoid-like EBA. Diagnosis of EBA can be challenging as it sharesclinical and histopathologic features with other blistering diseases. Treatment is often recalcitrant and will often necessitate multiple therapies. We presenta case of a thirty-six-year-old Chinese man with EBA and review the literature.

摘要

获得性大疱性表皮松解症(EBA)是一种罕见的获得性表皮下大疱性疾病。EBA的特征是针对Ⅶ型胶原蛋白的自身抗体,Ⅶ型胶原蛋白用于将表皮与真皮连接起来。EBA最常见的两种表现形式是经典非炎症性EBA和类大疱性类天疱疮EBA。EBA的诊断具有挑战性,因为它与其他大疱性疾病具有共同的临床和组织病理学特征。治疗往往难以奏效,通常需要多种治疗方法。我们报告一例36岁患有EBA的中国男性病例并复习相关文献。

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