Cho H J, Lee I J, Kim S C
Department of Dermatology, Yonsei University College of Medicine, Seoul, Korea.
Yonsei Med J. 1998 Aug;39(4):339-44. doi: 10.3349/ymj.1998.39.4.339.
Epidermolysis bullosa acquisita (EBA) is an autoimmune-mediated subepidermal bullous disease in which the target of the autoantibodies is type VII collagen, a major component of anchoring fibrils. The purpose of this study was to evaluate the complement-fixing abilities and IgG subclass distribution of autoantibodies in EBA, and to also attempt to investigate the relation between inflammation, complement fixation and IgG subclass distribution in EBA patients. Only 2 sera of 18 patients (11%) showed weak complement-fixing abilities. IgG1 and IgG4 were the most frequently and intensely stained IgG subclasses in EBA sera. We could not find any relationship between the clinico-pathologic types, complement-fixing abilities and IgG subclasses in EBA. These results suggested that complement activation may not be a key factor of bulla formation in EBA.
获得性大疱性表皮松解症(EBA)是一种自身免疫介导的表皮下大疱性疾病,其中自身抗体的靶标是VII型胶原蛋白,它是锚定原纤维的主要成分。本研究的目的是评估EBA中自身抗体的补体固定能力和IgG亚类分布,并尝试研究EBA患者炎症、补体固定和IgG亚类分布之间的关系。18例患者中只有2份血清(11%)显示出较弱的补体固定能力。IgG1和IgG4是EBA血清中最常且染色最强的IgG亚类。我们在EBA的临床病理类型、补体固定能力和IgG亚类之间未发现任何关系。这些结果表明补体激活可能不是EBA中水疱形成的关键因素。