Identification of the skin basement-membrane autoantigen in epidermolysis bullosa acquisita.
Identification of the skin basement-membrane autoantigen in epidermolysis bullosa acquisita.
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大疱性表皮松解症中皮肤基底膜自身抗原的鉴定。
DOI：
10.1056/nejm198404193101602
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发表时间：
1984
期刊：
影响因子：
--
通讯作者：
Gammon,WR
中科院分区：
文献类型：
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作者：
Woodley,DT;Briggaman,RA;O'Keefe,EJ;Inman,AO;Queen,LL;Gammon,WR
获得性大疱性表皮松解症是一种获得性皮肤慢性水泡性疾病，皮肤分离发生在表皮和真皮之间的基底膜区域。有证据表明，水泡是由免疫过程启动的。使用9名患者的血清样本作为抗体来源，我们已经确定了人类皮肤基底膜的一种主要蛋白质，它是这种疾病中自身抗体的抗原(或靶点)。这种以前未知的蛋白质由29万道尔顿和14.5万道尔顿两种成分组成，与基底膜的其他已知成分不同。这些研究证明获得性大疱性表皮松解症是一种特殊的疾病，不同于其他原发大疱性疾病，如大疱性类天疱疮和寻常型天疱疮，并提示已发现的基底膜成分可能在正常的表皮-真皮粘连中起作用。(n Engl J Med 1984；310：1007-13)
Epidermolysis bullosa acquisita is an acquired chronic blistering disease of the skin, in which separation of the skin occurs in the basement-membrane zone between the epidermis and the dermis. There is evidence that blistering is initiated by an immune process.Using serum samples from nine patients as a source of antibodies, we have identified a major protein of the basement membrane of human skin that serves as the antigen (or target) for autoantibodies in this disorder. This previously unrecognized protein, which consists of two components of 290,000 and 145,000 daltons, is distinct from other known components of the basement membrane. These studies provide evidence that epidermolysis bullosa acquisita is a specific disease that is different from other primary bullous diseases, such as bullous pemphigoid and pemphigus vulgaris, and suggest that the basement-membrane component that has been identified may have a role in normal epidermal–dermal adherence. (N Engl J Med 1984; 310:1007–13.)