Department of Dermatology and Allergology, Philipps University Deutschhausstrasse 9, D-35033 Marburg, Germany.
Clin Immunol. 2010 Apr;135(1):99-107. doi: 10.1016/j.clim.2009.12.010. Epub 2010 Jan 25.
Epidermolysis bullosa acquisita (EBA) is a severe immunobullous disease and is caused by IgG against type VII collagen (Col VII) of anchoring fibrils. In this study, utilizing ELISA and immunoblot, 13/15 EBA sera but 0/20 bullous pemphigoid sera and 0/30 healthy control sera showed IgG reactivity with distinct recombinant subregions of the non-collagenous domain 1 (NC1) of Col VII. In two EBA patients, IgG titers against Col VII-NC1 were grossly correlated to clinical disease activity. Moreover, Col VII-reactive T cells were identified in a representative EBA patient which recognized identical subdomains of Col VII-NC1. These findings strongly suggest that (1) the Col VII-NC1 ELISA is a powerful tool for making the diagnosis of EBA, (2) Col VII-specific IgG grossly relates to disease activity and (3) IgG reactivity is associated with T cell recognition of identical subdomains of Col VII-NC1.
获得性大疱性表皮松解症（EBA）是一种严重的免疫性大疱病，由针对锚纤维 VII 型胶原（Col VII）的 IgG 引起。在这项研究中，利用 ELISA 和免疫印迹法，13/15 例 EBA 血清但 0/20 例大疱性类天疱疮血清和 0/30 例健康对照组血清显示出与 Col VII 的非胶原域 1（NC1）的不同重组亚区的 IgG 反应性。在两名 EBA 患者中，针对 Col VII-NC1 的 IgG 滴度与临床疾病活动度有很大的相关性。此外，在一位代表性的 EBA 患者中鉴定出 Col VII 反应性 T 细胞，其识别 Col VII-NC1 的相同亚结构域。这些发现强烈表明：（1）Col VII-NC1 ELISA 是诊断 EBA 的有力工具，（2）Col VII 特异性 IgG 与疾病活动度有很大关系，（3）IgG 反应性与 Col VII-NC1 相同亚结构域的 T 细胞识别有关。
