BULLOUS SLE - A PHENOTYPICALLY DISTINCTIVE BUT IMMUNOLOGICALLY HETEROGENEOUS BULLOUS DISORDER
BULLOUS SLE - A PHENOTYPICALLY DISTINCTIVE BUT IMMUNOLOGICALLY HETEROGENEOUS BULLOUS DISORDER
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DOI：
10.1038/jid.1993.20
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发表时间：
1993-01-01
影响因子：
6.5
通讯作者：
BRIGGAMAN, RA
中科院分区：
文献类型：
--
作者：
GAMMON, WR;BRIGGAMAN, RA
大疱性系统性红斑狼疮(SLE)是一种罕见的水泡性疾病，具有独特的临床、组织学和免疫病理特征，共同构成独特的大疱性疾病表型。大疱性系统性红斑狼疮至少有两种不同的免疫学亚型，其特征是存在或不存在识别III型胶原的循环和/或组织结合的基底膜带自身抗体。除了间接免疫荧光和/或直接免疫电子显微镜检查外，这两种亚型不能明确区分。在没有循环抗体的患者中，需要免疫电子显微镜来区分这两种亚型。自身抗体VII型胶原的患者与获得性大疱性表皮松解症患者相似，但不完全相同，大疱性表皮松解症是另一种与VII型胶原自身抗体相关的大疱性疾病。大疱性SLE患者的VII型胶原自身抗体只是表明对该蛋白的自身免疫和对SLE的易感性是相关现象的几条证据之一。此外，有新的证据表明大疱性获得性表皮松解症与系统性红斑狼疮之间存在关联。也有证据表明，大疱性系统性红斑狼疮(和获得性大疱性表皮松解症)中存在针对VII型胶原的自身抗体，它们的产生受第二类主要组织相容性复合体DRβ1等位基因1501和其他DRβ1等位基因的调控，这些等位基因在第二高变区具有相似的氨基酸序列。
Bullous systemic lupus erythematosus (SLE) is a rare blistering disease with a distinctive combination of clinical, histologic and immunopathologic features that together constitute a unique bullous disease phenotype. There appear to be at least two immunologically distinct subtypes of bullous SLE characterized by the presence or absence of circulating and/or tissue-bound basement membrane zone autoantibodies that recognize type VII collagen. The two subtypes are not clearly distinguishable except by indirect immunofluorescence and/or direct immunoelectron microscopy. In patients without circulating antibodies, immunoelectron microscopy is required to distinguish between the two subtypes. Patients with autoantibodies to me VII collagen are similar but not identical to patients with epidermolysis bullosa acquisita-another bullous disease associated with autoantibodies to type VII collagen. Autoantibodies to type VII collagen in patients with bullous SLE is only one of several lines of evidence that indicate autoimmunity to that protein and susceptibility to SLE are associated phenomena. In addition, there is emerging evidence for an association between epidermolysis bullous acquisita and SLE. There is also evidence that autoantibodies to type VII collagen are pathogenic in bullous SLE (and epidermolysis bullosa acquisita) and that their production is regulated by the class II major histocompatibility complex DR beta 1 allele, 1501 and possibly other DR beta 1 alleles that share a similar sequence of amino acids in the second hypervariable region.