Kumar Vinod, De Dipankar, Gupta Smriti, Narayan R Vignesh, Mahajan Rahul, Chatterjee Debajyoti, Handa Sanjeev
Department of Dermatology, Venerology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Indian J Dermatol Venereol Leprol. 2025 May-Jun;91(3):300-304. doi: 10.25259/IJDVL_1195_2023.
Background Autoimmune blistering disorders (AIBD) result from the formation of auto-antibodies against adhesion proteins of the skin/mucosa(e). These auto-antibodies can be detected in the bound form in the tissue using direct immunofluorescence (DIF) or blood circulation using enzyme-linked immunosorbent assay (ELISA) or other methods. Objectives The objective of this study was to evaluate the concordance rate between the results of multivariant ELISA and the diagnosis of AIBD made using DIF and histopathology in an appropriate clinical context. Methods This was a retrospective study (December 2020 to April 2023) in which the multivariant ELISA assay (able to detect antibodies against desmoglein 1, desmoglein 3, BP180, BP230, envoplakin, and collagen VII) data were retrieved from the dermatology laboratory. Corresponding clinical and histopathology data were searched from relevant institutional databases. As per routine practice, the final diagnosis was assigned based on the clinical presentation, histopathology features and corresponding DIF report. Results After screening the records of 338 patients during the study period, 253 patients were included. Of them, 194 had AIBD and 59 had non-AIBD. In the autoimmune blistering disorder group, 122 and 72 patients had pemphigus and pemphigoid, respectively. Overall, a good level of agreement was found between multivariant ELISA results and the final diagnosis (Fleiss kappa = 0.631, p-value < 0.001). The pemphigus vulgaris group exhibited good agreement (kappa = 0.796, p < 0.001), while pemphigus foliaceous, bullous pemphigoid and non-autoimmune blistering disorders demonstrated moderate agreement (kappa = 0.641, 0.651, 0.533, respectively; p < 0.001). The mucous membrane pemphigoid group had a fair agreement (kappa = 0.289; p < 0.001). Limitations The limitations for the study were its retrospective design, fewer number of patients in certain groups like paraneoplastic pemphigus and gold-standard single antigen specific ELISA was not done. Conclusion Considering good agreement between the multivariant ELISA and the gold-standard diagnosis (clinical findings plus histopathology plus DIF), multivariant ELISA can be used for the diagnosis of AIBDs in places where facilities for DIF are unavailable. Multivariant ELISA can improve etiological diagnosis for a set of autoimmune blistering disorders whose target antigens are represented in the multivariant panel.
背景 自身免疫性水疱病（AIBD）是由针对皮肤/黏膜黏附蛋白的自身抗体形成所致。这些自身抗体可通过直接免疫荧光法（DIF）在组织中以结合形式检测到，或通过酶联免疫吸附测定（ELISA）或其他方法在血液循环中检测到。目的 本研究的目的是在适当的临床背景下，评估多变量ELISA结果与使用DIF和组织病理学做出的AIBD诊断之间的符合率。方法 这是一项回顾性研究（2020年12月至2023年4月），从皮肤科实验室检索多变量ELISA检测（能够检测抗桥粒芯糖蛋白1、桥粒芯糖蛋白3、BP180、BP230、内披蛋白和Ⅶ型胶原的抗体）数据。从相关机构数据库中搜索相应的临床和组织病理学数据。按照常规做法，根据临床表现、组织病理学特征和相应的DIF报告做出最终诊断。结果 在研究期间筛选了338例患者的记录后，纳入了253例患者。其中，194例患有AIBD，59例患有非AIBD。在自身免疫性水疱病组中，分别有122例和72例患者患有天疱疮和类天疱疮。总体而言，多变量ELISA结果与最终诊断之间存在良好的一致性（Fleiss卡方 = 0.631，p值 < 0.001）。寻常型天疱疮组表现出良好的一致性（卡方 = 0.796，p < 0.001），而落叶型天疱疮、大疱性类天疱疮和非自身免疫性水疱病表现出中等一致性（卡方分别为0.641、0.651、0.533；p < 0.001）。黏膜类天疱疮组一致性一般（卡方 = 0.289；p < 0.001）。局限性 本研究的局限性在于其回顾性设计，某些组（如副肿瘤性天疱疮）患者数量较少，且未进行金标准单抗原特异性ELISA。结论 考虑到多变量ELISA与金标准诊断（临床发现加组织病理学加DIF）之间的良好一致性，在没有DIF检测设施的地方，多变量ELISA可用于AIBD的诊断。多变量ELISA可改善对一组靶抗原包含在多变量检测板中的自身免疫性水疱病的病因诊断。
