IgG4是妊娠性类天疱疮的主要IgG亚型  

IgG4 as the predominant Ig Gsubclass in pemphigoides gestationis

作　　者：Patton T. Plunkett R.W. BeutnerE.H. D.M. Jukic 冯义国 

机构地区：[1]Dermatopathology Unit, Shadyside Hospital, University of Pittsburgh Medical Center, Centre Avenue, Pittsburgh, PA, United States Dr.

出　　处：《世界核心医学期刊文摘（皮肤病学分册）》2006年第6期61-62,共2页Digest of the World Core Medical JOurnals:Dermatology

摘　　要：Background: Pemphigoides gestationis (PG) is a blistering disorder of pregnancy caused by antibodies against basement membrane proteins. They are directed against the 180 kD bullous pemphigoid antigen (BPAg2), towards the epitopes within the NC 16A domain. There are many similarities between pemphigoid gestationis and bullous pemphigoid (BP), but the literature so far indicated different immunofluorescence results in regards with C3 and IgG, and IgG subclasses (IgG4 vs. IgG1). Methods: We evaluated staining patterns and IgG subclasses, as well as C5b-9 membrane attack complex (MAC) in 10 pregnant patients with PG, using sandwich double antibody immunofluorescence (SDAI) and direct immunofluorescence (DIF). Results: All ten specimens stained with C3 by DIF, but only five had trace amount of IgG reactants by this method. By SDAI, 100%were positive for the IgG4 and C5b-9 MAC, 70%for IgG2, 50%for IgG1, and 40%for IgG3. Conclusion: IgG4 was the predominant IgG subtype identified. This finding has not been reported for PG, but it mimics results reported for BP. One explanation is prolonged disease course, as well as blocking of antigenic domains by IgG4. Understanding this completely will help develop therapies and prevention strategies for immunobullous and other autoimmune diseases, and perhaps aid in an exact classification.Background： Pemphigoides gestationis （PG） is a blistering disorder of pregnancy caused by antibodies against basement membrane proteins. They are directed against the 180 kD bullous pemphigoid antigen （BPAg2）, towards the epitopes within the NC 16A domain. There are many similarities between pemphigoid gestationis and bullous pemphigoid （BP）, but the literature so far indicated different immunofluorescence results in regards with C3 and IgG, and IgG subclasses （IgG4 vs. IgG1）. Methods： We evaluated staining patterns and IgG subclasses, as well as C5b- 9 membrane attack complex （MAC） in 10 pregnant patients with PG, using sandwich double antibody immunofluorescence （SDAI） and direct immunofluorescence （DIF） . Results： All ten specimens stained with C3 by DIF, but only five had trace amount of IgG reactants by this method. By SDAI, 100% were positive for the IgG4 and C5b- 9 MAC, 70% for IgG2, 50% for IgG1, and 40% for IgG3. Conclusion： IgG4 was the predominant IgG subtype identified. This finding has not been reported for PG, but it mimics results reported for BP. One explanation is prolonged disease course, as well as blocking of antigenic domains by IgG4. Understanding this completely will help develop therapies and prevention strategies for immunobullous and other autoimmune diseases, and perhaps aid in an exact classification.

关 键 词：大疱性类天疱疮 IGG亚型 IGG4 妊娠性 直接免疫荧光技术 自身免疫性疾病 基底膜蛋白 C5B-9 双抗体 检测分析 

分 类 号：R758.66[医药卫生—皮肤病学与性病学] R746.103[医药卫生—临床医学]