Autoimmunity to type VII collagen: epidermolysis bullosa acquisita.
Autoimmunity to type VII collagen: epidermolysis bullosa acquisita.
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对 VII 型胶原蛋白的自身免疫：获得性大疱性表皮松解症。
DOI：
10.1159/000131455
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发表时间：
2008
期刊：
影响因子：
--
通讯作者：
D. Woodley
中科院分区：
文献类型：
--
作者：
J. Remington;Mei Chen;Julie Burnett;D. Woodley
获得性大疱性表皮病（EBA）是一种获得性的机械性大疱性疾病，其特征是对VII型胶原的自身免疫。VII型胶原蛋白形成锚定纤维，将表皮及其下面的基底膜区连接到乳头状真皮的结构。EBA患者表现出皮肤脆性、水疱、疤痕和粟粒形成，使人联想到遗传性营养不良性大疱性表皮病（DEB）。DEB患者由于编码VII型胶原蛋白的基因中的遗传缺陷而具有微小的或不存在的锚定原纤维。EBA患者的正常功能的锚定原纤维减少，继发于免疫系统异常，其中它们产生“致病性”IgG抗VII型胶原抗体。这些自身抗体的致病性已经通过被动转移动物模型证明，其中将抗VII型胶原抗体注射到小鼠中在动物中产生EBA样起泡疾病。EBA有几种不同的临床表现。它可以表现出与DEB、大疱性类天疱疮、瘢痕性类天疱疮、Brunsting-Perry类天疱疮或伊加大疱性皮肤病相似的特征。EBA的治疗并不令人满意，然而，秋水仙碱、氨苯砜、氨氯地平、英夫利昔单抗和静脉注射免疫球蛋白已报告了一些治疗成功。
Epidermolysis bullosa acquisita (EBA) is an acquired, mechanobullous disease characterized by autoimmunity to type VII collagen. Type VII collagen makes anchoring fibrils, structures that connect the epidermis and its underlying basement membrane zone to the papillary dermis. EBA patients exhibit skin fragility, blisters, scars and milia formation reminiscent of genetic dystrophic epidermolysis bullosa (DEB). DEB patients have diminutive or absent anchoring fibrils due to a genetic defect in the gene encoding type VII collagen. EBA patients have a decrease in normally functioning anchoring fibrils secondary to an abnormality in their immune system in which they produce 'pathogenic' IgG anti-type VII collagen antibodies. The pathogenicity of these autoantibodies has been demonstrated by passive transfer animal models, in which anti-type VII collagen antibodies injected into a mouse produced an EBA-like blistering disease in the animal. EBA has several distinct clinical presentations. It can present with features similar to DEB, bullous pemphigoid, cicatricial pemphigoid, Brunsting-Perry pemphigoid or IgA bullous dermatosis. Treatment for EBA is unsatisfactory, however, some therapeutic success has been reported with colchicine, dapsone, photophoresis, infliximab and intravenous immunoglobulin.