Epidermolysis bullosa acquisita: a disease of autoimmunity to type VII collagen.
Epidermolysis bullosa acquisita: a disease of autoimmunity to type VII collagen.
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大疱性表皮松解症：一种针对 VII 型胶原的自身免疫性疾病。
DOI：
10.1016/0896-8411(91)90007-y
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发表时间：
1991
影响因子：
12.8
通讯作者：
Gammon,WR
中科院分区：
文献类型：
--
作者：
Gammon,WR
获得性大疱性表皮松解症（EBA）是一组原发性水疱疾病之一，其特征是复层鳞状上皮基底膜（BM）的真皮/表皮分离和IgG抗BM自身抗体（ABM）（表1和2）。EBA ABM可以通过其与BM基质蛋白、VII型胶原（C-VII）的反应性而与所有其他原发性起泡疾病中的IgG ABM区分开。EBA的临床和组织学特征是高度可变的，可能无法与其他原发性大疱性疾病相区分。诊断可以通过直接或间接证明IgG ABM到C-VII的几种方法之一来证实。IgG ABM至C-VII对EBA无特异性。在伴或不伴继发性水疱性皮疹（SLE大疱性皮疹（BSLE））的SLE患者中均有描述。伊加ABMs到C-VII已经在线性伊加大疱性皮肤病患者亚组中描述。最近的证据表明，C-VII的自身免疫EBA和BSLE的主要组织相容性复合体（MHC）内的相同的基因调节，并有助于急性炎症和水泡的发病机制，在这两种疾病。在SLE和BSLE中发现C-VII的ABM，证据表明EBA和BSLE具有C-VII自身免疫的免疫遗传易感性，以及SLE易感性与EBA之间的明显关联表明SLE与C-VII自身免疫密切相关。
Epidermolysis bullosa acquisita (EBA) is one of a group of primary blistering diseases characterized by dermal/epidermal separation at the basement membrane (BM) of stratified squamous epithelium and IgG anti-BM autoantibodies (ABM) (Tables 1 and 2). EBA ABMs can be distinguished from IgG ABMs in all other primary blistering diseases by their reactivity with the BM matrix protein, type VII collagen (C-VII). The clinical and histological features of EBA are highly variable and may be indistinguishable from those of other primary bullous diseases. The diagnosis can be confirmed by one of several methods that directly or indirectly demonstrate IgG ABMs to C-VII. IgG ABMs to C-VII are not specific for EBA. They have been described in SLE patients with and without a secondary blistering eruption, bullous eruption of SLE (BSLE). IgA ABMs to C-VII have been described in a subgroup of patients with linear IgA bullous dermatosis. Recent evidence suggests that autoimmunity to C-VII in EBA and BSLE is regulated by the same genes within the major histocompatibility complex (MHC) and contributes to the pathogenesis of acute inflammation and blisters in both disorders. The finding of ABMs to C-VII in SLE and BSLE, evidence that EBA and BSLE share an immunogenetic predisposition to C-VII autoimmunity, and an apparent association between susceptibility to SLE and EBA suggest a close relationship between SLE and autoimmunity to C-VII.