Characteristics of IgG subclasses and complement deposition in BP230-type bullous pemphigoid
Characteristics of IgG subclasses and complement deposition in BP230-type bullous pemphigoid
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BP230型大疱性类天疱疮IgG亚类及补体沉积特征
DOI：
10.1111/jdv.15325
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发表时间：
2018
影响因子：
9.2
通讯作者：
Shimizu H.
中科院分区：
文献类型：
--
作者：
Zheng M.;Ujiie H.;Iwata H.;Muramatsu K.;Yoshimoto N.;Ito T.;Ujiie I.;Shimizu S.;Sato-Matsumura K.C.;Shimizu H.
大疱性类天疱疮（BP）是最常见的自身免疫性水疱疾病。BP180是BP的主要自身抗原，在部分BP病例中，BP230是自身抗体的唯一靶标。这样的血压被称为BP230型血压。BP230型BP往往表现出比常规BP更温和的临床表型，但原因尚不清楚。自身抗体和补体活化的致病作用已在常规BP中得到证实，但IgG亚类的分布和补体沉积在BP230‐型BP中的程度尚不清楚。目的比较BP180和BP230自身抗体（BP180 - BP230 - type BP）在BP230型BP和常规BP中的IgG亚类分布和补体沉积程度。方法根据病变组织病理学、病变周围皮肤IgG和补体的沉积以及循环中BP180和BP230自身抗体的存在，证实BP的诊断。用大疱性类天疱疮疾病面积指数判断病情严重程度。采用直接免疫荧光法检测6例BP230型和11例BP180 - BP230型BP患者病灶周围皮肤IgG亚类沉积和补体沉积。结果67%的BP230型BP患者表现为轻度临床表型。所有BP230型BP病例和82%的BP180 - BP230型BP病例在皮肤基底膜区发现IgG4明显沉积。值得注意的是，在所有BP230型BP病例中，IgG1和IgG3的沉积都是微弱的或阴性的，而在BP180 - BP230型BP病例中，IgG1和IgG3的沉积分别为91%和64%。补体C3在BP230型BP中的沉积倾向于弱于BP180 - BP230型BP。结论BP230型BP的轻度临床表型可能与皮肤病变部位IgG1、IgG3和补体沉积较弱有关。
BackgroundBullous pemphigoid (BP) is the most common autoimmune blistering disease. BP180 is the primary autoantigen of BP, and in a portion of BP cases, BP230 is the only target of autoantibodies. Such BP is called BP230‐type BP. BP230‐type BP tends to show milder clinical phenotypes than conventional BP, but the reason is unclear. The pathogenic roles of autoantibodies and complement activation have been shown in conventional BP, but the distribution of IgG subclasses and the degree of complement deposition in BP230‐type BP remain unclear.ObjectiveTo compare the distribution of IgG subclasses and the degree of complement deposition in BP230‐type BP with those in conventional BP with autoantibodies to BP180 and BP230 (BP180‐BP230‐type BP).MethodsThe diagnosis of BP was confirmed by the histopathology of the lesions, the deposition of IgG and complement in the perilesional skin and the presence of circulating autoantibodies to BP180 and BP230. The disease severity was determined by bullous pemphigoid disease area index. The deposition of IgG subclasses and complement deposition were examined by direct immunofluorescence of the perilesional skin in 6 BP230‐type BP cases and 11 BP180‐BP230‐type BP cases.ResultsSixty seven percent of BP230‐type BP cases show a mild clinical phenotype. All BP230‐type BP cases and 82% of BP180‐BP230‐type BP cases were found to demonstrate the clear deposition of IgG4 at the basement membrane zone of skin specimens. Notably, the deposition of IgG1 and IgG3 was faint or negative in all of the BP230‐type BP cases, whereas they were clearly detected in 91% and 64% of the BP180‐BP230‐type BP cases, respectively. The deposition of complement C3 tended to be weaker in BP230‐type BP than in BP180‐BP230‐type BP.ConclusionThe mild clinical phenotype of BP230‐type BP may correlate with the weaker deposition of IgG1, IgG3 and complement in the skin lesions.