Epidermolysis bullosa acquisita: What's new?
Epidermolysis bullosa acquisita: What's new?
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DOI：
10.1111/j.1346-8138.2009.00799.x
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发表时间：
2010-03-01
影响因子：
3.1
通讯作者：
Hashimoto, Takashi
中科院分区：
文献类型：
--
作者：
Ishii, Norito;Hamada, Takahiro;Hashimoto, Takashi
VII型胶原是上皮基底膜中细胞外基质的粘附分子，并且是真皮-表皮连接（DEJ）处的锚定原纤维的主要成分。针对这种蛋白质的自身免疫引起罕见的器官特异性获得性大疱性表皮病（EBA）。EBA是一种罕见的获得性、异质性、慢性皮肤和粘膜水疱性疾病，其特征在于表皮下水疱和组织结合以及循环的DEJ自身抗体。EBA与其他表皮下大疱性疾病（如主要为营养不良性大疱性表皮病或大疱性类天疱疮）有几种不同的临床表现。EBA的循环免疫球蛋白G自身抗体与290 kDa真皮蛋白VII型胶原反应，如使用真皮提取物通过免疫印迹分析检测到的。这些自身抗体的致病性已经通过实验动物模型证明，其中将抗VII型胶原抗体注射到小鼠中在动物中产生EBA样水疱疾病。EBA病例通常需要高剂量的全身性皮质类固醇和各种免疫抑制剂。虽然EBA的治疗通常困难且不令人满意，但已报道了秋水仙碱、氨苯砜、英夫利昔单抗和静脉注射免疫球蛋白的一些治疗成功。本文就EBA的临床表现、发病机制及治疗等方面的最新进展作一综述。
Type VII collagen is an adhesion molecule of the extracellular matrix in epithelial basement membranes, and the main constituent of anchoring fibrils at the dermal-epidermal junction (DEJ). Autoimmunity against this protein is causing the rare organ-specific epidermolysis bullosa acquisita (EBA). EBA is a rare acquired, heterogeneous, chronic blistering disease of skin disease of skin and mucous membranes characterized by subepidermal blisters and tissue-bound as well as circulating autoantibodies to the DEJ. EBA has several distinct clinical presentations with other subepidermal bullous diseases, such as mainly dystrophic epidermolysis bullosa or bullous pemphigoid. The circulating immunoglobulin G autoantibodies for EBA react with a 290-kDa dermal protein, type VII collagen, as detected by immunoblot analysis using dermal extracts. The pathogenicity of these autoantibodies has been demonstrated by experimental animal models, in which anti-type VII collagen antibodies injected into a mouse produced an EBA-like blistering disease in the animal. EBA cases often require high doses of systemic corticosteroids and a variety of immunosuppressants. Although treatment for EBA is frequently difficult and unsatisfactory, some therapeutic success has been reported with colchicine, dapsone, infliximab and i.v. immunoglobulin. In this review, we will focus on recent progress in our understanding of the clinical manifestations, the etiopathogenesis as well as the management of EBA.