Department of Dermatology, The Keck School of Medicine at the University of Southern California, Los Angeles, CA 90033, USA.
Autoimmunity. 2012 Feb;45(1):91-101. doi: 10.3109/08916934.2011.606450. Epub 2011 Sep 28.
Epidermolysis bullosa acquisita (EBA) is a rare and acquired autoimmune subepidermal bullous disease of skin and mucosa. EBA includes various distinct clinical manifestations resembling genetic dystrophic epidermolysis bullosa (DEB), Bullous pemphigus, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. These patients have autoantibodies against type VII collagen (C7), an integral component of anchoring fibrils (AFs), which are responsible for attaching the dermis to the epidermis. Destruction or perturbation of the normal functioning AFs clinically results in skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic epidermolysis bullosa. These anti-C7 antibodies are "pathogenic" because when injected into a mouse, the mouse develops an EBA-like blistering disease. Currently, treatment is often unsatisfactory; however, some success has been achieved with colchicine, dapsone, photopheresis, plasmapheresis, infliximab, rituximab, and IVIG.
获得性大疱性表皮松解症（EBA）是一种罕见的获得性自身免疫性表皮下大疱性皮肤病和黏膜病。EBA 包括各种不同的临床表现，类似于遗传性营养不良性大疱性表皮松解症（DEB）、大疱性类天疱疮、Brunsting-Perry 类天疱疮或瘢痕性类天疱疮。这些患者自身抗体针对 VII 型胶原（C7），C7 是锚定纤维（AFs）的重要组成部分，负责将真皮与表皮连接。正常功能的 AFs 的破坏或紊乱在临床上导致皮肤脆弱、水疱、糜烂、瘢痕、粟粒疹和指甲脱落，所有这些特征都类似于遗传性营养不良性大疱性表皮松解症。这些抗 C7 抗体是“致病性的”，因为当将它们注入小鼠体内时，小鼠会发展出类似于 EBA 的水疱病。目前，治疗往往不尽如人意；然而，秋水仙碱、氨苯砜、光化学疗法、血浆置换、英夫利昔单抗、利妥昔单抗和 IVIG 已取得一些成功。
