Simultaneous immunolocalization of desmoglein 3 and IgG4 in oral pemphigus vulgaris: IgG4 predominant autoantibodies in its pathogenesis.
Simultaneous immunolocalization of desmoglein 3 and IgG4 in oral pemphigus vulgaris: IgG4 predominant autoantibodies in its pathogenesis.
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口腔寻常型天疱疮中桥粒芯糖蛋白 3 和 IgG4 的同时免疫定位：IgG4 在其发病机制中占主导地位的自身抗体。
DOI：
10.1111/jop.12290
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发表时间：
2014
影响因子：
3.3
通讯作者：
Saku T
中科院分区：
文献类型：
--
作者：
Abe T;Maruyama S;Babkair H;Yamazaki M;Cheng J;Saku T
口腔寻常天疱疮（Oral Pemphigus vulgaris，PV）是一种自身免疫性水疱性疾病，主要由抗桥粒芯糖蛋白（Desmoglein，Dsg）3自身抗体介导.然而，在PV的诊断程序中，没有注意到针对Dsg3的自身抗体的IgG亚类。因此，我们在这项研究中的目的是调查Dsg3和任何IgG亚类是否在PV口腔粘膜的组织切片中化学共定位。材料和方法从9例PV病例的活检标本的福尔马林固定的石蜡块和手术切除的正常颊粘膜的活检标本中连续切片，比较检查Dsg3，IgG 4，结果Dsg3在正常和PV组织中均呈点状分布于上皮细胞边缘和整个上皮细胞胞浆中，而在PV的漂浮上皮细胞中呈不规则染色。IgG4也被证明是在一个间断的方式之间的浮动上皮片，这是几乎相同的Dsg3的免疫组化配置文件的细胞边界。除了在上皮部分中检测到之外，IgG 4信号显著地定位于散布在肉芽组织中的浆细胞中，其中IgG 4阳性（+）浆细胞与IgG+细胞的比率非常高（平均28%）这些结果首次证实了Dsg3自身抗体在口腔PV中主要由IgG 4组成，Dsg3和IgG4免疫组化在PV的组织病理学诊断中可能是有价值的辅助手段。
BackgroundOral pemphigus vulgaris (PV), an autoimmune blistering disease, is mainly mediated by autoantibodies against desmoglein (Dsg) 3. However, no attention has been paid to IgG subclasses of the autoantibodies against Dsg3 in the diagnostic procedure for PV. Thus, our aim in this study was to investigate whether Dsg3 and any of IgG subclasses are immunohistochemically colocalized in tissue sections of PV oral mucosa.Materials and MethodsSerial sections cut from formalin‐fixed paraffin blocks of biopsy specimens of 9 PV cases and those of normal buccal mucosa surgically removed for fibro‐epithelial polyps were comparatively examined for immunohistochemical localizations for Dsg3, IgG4, and IgG.ResultsDsg3 was demonstrated in a dot‐like pattern on the cell border and in the cytoplasm of the whole epithelial layer in both normal and PV specimens, while its staining was irregular among floating epithelial sheets of PV. IgG4 was also demonstrated in a punctuated fashion on the cell border among floating epithelial sheets, which was nearly identical to the immunohistochemical profile of Dsg3. In addition to being detected in the epithelial part, IgG4 signals were prominently localized in plasma cells scattered in the granulation tissue, where ratios of IgG4‐positive (+) plasma cells to IgG+ cells were extraordinarily higher (mean 28%) than those in normal mucosa.DiscussionThese findings confirmed for the first time that autoantibodies against Dsg3 are mainly composed of IgG4 in oral PV and that the combined immunohistochemistry for Dsg3 and IgG4 can be a valuable aid in confirming a histopathological diagnosis of PV.