Gammon W R, Heise E R, Burke W A, Fine J D, Woodley D T, Briggaman R A
Department of Dermatology, University of North Carolina, Chapel Hill 27514.
J Invest Dermatol. 1988 Sep;91(3):228-32. doi: 10.1111/1523-1747.ep12470317.
Epidermolysis bullosa acquisita (EBA) is a chronic blistering disease characterized by circulating and tissue bound IgG auto-antibodies to the basement membrane zone (BMZ) of stratified squamous epithelium. Recent studies have shown that antibodies recognize epitopes present in the noncollagenous carboxyl-terminal domain of type VII collagen, a BMZ matrix protein. Antibodies with identical specificity also have been detected in patients with the rare blistering disease, bullous systemic lupus erythematosus (bullous SLE), suggesting EBA and bullous SLE are immunologically related diseases. In this study we determined the major histo-compatibility antigen types of 29 EBA patients and 6 patients with bullous SLE. Analysis of the results showed HLA-DR2 was significantly increased in both black EBA patients, P = 0.013 (corrected, RR = 4.8) and white EBA patients, P = 0.0008 (corrected, RR = 13.1). Five of the six bullous SLE patients also were positive for the DR2 antigen, P = 0.009. These results show the expression of autoimmunity to type VII collagen is HLA class II allele associated and that EBA and bullous SLE are immunogenetically related diseases.
获得性大疱性表皮松解症（EBA）是一种慢性水疱性疾病，其特征是循环中和组织中存在针对复层鳞状上皮基底膜带（BMZ）的IgG自身抗体。最近的研究表明，这些抗体识别VII型胶原蛋白（一种BMZ基质蛋白）非胶原羧基末端结构域中存在的表位。在罕见的水疱性疾病大疱性系统性红斑狼疮（大疱性SLE）患者中也检测到了具有相同特异性的抗体，这表明EBA和大疱性SLE是免疫相关疾病。在本研究中，我们确定了29例EBA患者和6例大疱性SLE患者的主要组织相容性抗原类型。结果分析显示，HLA-DR2在黑人EBA患者中显著增加，P = 0.013（校正后，RR = 4.8），在白人EBA患者中也显著增加，P = 0.0008（校正后，RR = 13.1）。6例大疱性SLE患者中有5例DR2抗原也呈阳性，P = 0.009。这些结果表明，针对VII型胶原蛋白的自身免疫表达与HLA II类等位基因相关，并且EBA和大疱性SLE是免疫遗传学相关疾病。
