Department of Dermatology and Cutaneous Biology Research Institute, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
J Eur Acad Dermatol Venereol. 2013 Oct;27(10):1204-13. doi: 10.1111/jdv.12096. Epub 2013 Feb 1.
Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune subepidermal bullous disease with clinical features similar to the genetic form of dystrophic epidermolysis bullosa. EBA is characterized by the presence of autoantibodies against type VII collagen which is a major component of the anchoring fibrils at the dermal-epidermal junction. EBA can be divided into two main clinical types; mechanobullous and inflammatory EBA. Mechanobullous EBA, referred to as classic EBA, presents with skin fragility, blisters and dystrophic changes on trauma-prone areas. Inflammatory EBA resembles other autoimmune subepidermal bullous diseases. Compelling evidence from mouse models supports a pathogenic role of autoantibodies against type VII collagen in EBA. Treatment of EBA is often unsatisfactory. The most widely used systemic treatment is corticosteroids. Colchicine and dapsone have been reported to be good treatment modalities when combined with corticosteroids. Some intractable cases of EBA have successfully been treated with intravenous immunoglobulin or rituximab.
获得性大疱性表皮松解症（EBA）是一种慢性自身免疫性表皮下大疱性疾病，其临床特征与遗传性营养不良性大疱性表皮松解症相似。EBA 的特征是存在针对 VII 型胶原的自身抗体，VII 型胶原是真皮-表皮连接处锚定纤维的主要成分。EBA 可分为两种主要的临床类型；机械性大疱性和炎症性 EBA。机械性大疱性 EBA，称为经典 EBA，表现为皮肤脆弱、易受伤部位出现水疱和营养不良性改变。炎症性 EBA 类似于其他自身免疫性表皮下大疱性疾病。来自小鼠模型的有力证据支持自身抗体针对 VII 型胶原在 EBA 中的致病作用。EBA 的治疗往往不尽如人意。最广泛使用的系统治疗是皮质类固醇。秋水仙碱和氨苯砜与皮质类固醇联合使用已被报道为良好的治疗方法。一些难治性 EBA 病例成功地接受了静脉注射免疫球蛋白或利妥昔单抗治疗。
