Brooks W S, Lee Y Y, Abell E, Deng J S
Department of Dermatology, University of Pittsburgh School of Medicine, Pennsylvania.
J Clin Lab Anal. 1989;3(5):307-11. doi: 10.1002/jcla.1860030509.
Bullous pemphigoid and pemphigus are autoimmune disorders of skin of unknown etiology and are characterized by the presence of immunoreactants in the skin and circulating autoantibodies to skin components. The distribution of IgG subclass antibodies to intercellular substance (ICS) of pemphigus and basement membrane zone substance (BMZ) of bullous pemphigoid was analyzed by using monoclonal antibodies to human IgG subclasses. IgG4 type anti-BMZ antibody was found in the majority of patients with bullous pemphigoid (88% in skin and 96% in serum). One third to one half of bullous pemphigoid patients had IgG1 and IgG2 anti-BMZ antibodies. The majority of bullous pemphigoid skin (92%) had complement in skin, however only one third of their sera had complement binding activity in vitro. IgG1 anti-ICS antibody was the predominant one in patients with pemphigus (86% in skin and 80% in circulation). IgG4 anti-ICS antibody was seen in two thirds of specimens from pemphigus patients. IgG3 subclass antibody was more frequently seen in pemphigus than in bullous pemphigoid patients. Two-thirds of pemphigus sera were capable of activating complement in vitro. The complement binding activity was directly associated with IgG1 and/or IgG3 subclass antibodies. The possible mechanisms for the restricted IgG4 subclass antibodies in bullous pemphigoid and pemphigus are discussed.
大疱性类天疱疮和天疱疮是病因不明的皮肤自身免疫性疾病，其特征是皮肤中存在免疫反应物以及循环中存在针对皮肤成分的自身抗体。通过使用针对人IgG亚类的单克隆抗体，分析了天疱疮细胞间物质（ICS）和大疱性类天疱疮基底膜带物质（BMZ）的IgG亚类抗体分布。在大多数大疱性类天疱疮患者中发现了IgG4型抗BMZ抗体（皮肤中为88%，血清中为96%）。三分之一至二分之一的大疱性类天疱疮患者有IgG1和IgG2抗BMZ抗体。大多数大疱性类天疱疮皮肤（92%）中有补体，但只有三分之一的患者血清在体外具有补体结合活性。IgG1抗ICS抗体是天疱疮患者中的主要抗体（皮肤中为86%，循环中为80%）。在三分之二的天疱疮患者标本中可见IgG4抗ICS抗体。IgG3亚类抗体在天疱疮患者中比在大疱性类天疱疮患者中更常见。三分之二的天疱疮血清在体外能够激活补体。补体结合活性与IgG1和/或IgG3亚类抗体直接相关。讨论了大疱性类天疱疮和天疱疮中IgG4亚类抗体受限的可能机制。
