Immunoglobulin M pemphigoid
Immunoglobulin M pemphigoid
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DOI：
10.1016/j.jaad.2021.01.017
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发表时间：
2021-11-18
影响因子：
13.8
通讯作者：
Schmidt, Enno
中科院分区：
文献类型：
--
作者：
Boch, Katharina;Hammers, Christoph M.;Schmidt, Enno
背景：类天疱疮疾病是一种异质性的自身免疫性水疱疾病，其特征是主要沉积免疫球蛋白G或免疫球蛋白a自身抗体对抗皮表皮交界处（DEJ）结构蛋白。在类天疱疮疾病中，在DEJ观察到唯一的线性免疫球蛋白M （IgM）沉积；然而，igm特异性靶抗原尚未确定。目的：探讨IgM类天疱疮患者的特征。方法：采用组织病理学、直接和间接免疫荧光显微镜、酶联免疫吸附法、免疫印迹法、冷冻切片法、补体固定试验和内化试验对igm阳性患者的皮肤活检标本和血清进行评估。结果：沿DEJ检测到组织结合的线性IgM沉积和循环的抗XVII型胶原（Col17）的IgM自身抗体。这些循环IgM自身抗体没有补体激活或水疱诱导能力，但具有Col17体内内化的能力。局限性：患者数量有限。结论：本研究为IgM自身抗体在类天疱疮疾病中的作用提供了进一步的证据，并强调Col17是IgM类天疱疮的靶抗原。
Background: Pemphigoid diseases are a heterogeneous group of autoimmune blistering disorders characterized by predominant deposition of immunoglobulin G or immunoglobulin A autoantibodies against structural proteins of the dermoepidermal junction (DEJ). Sole linear immunoglobulin M (IgM) deposits at the DEJ in pemphigoid diseases have been observed; however, IgM-specific target antigens have not been identified. Objective: Characterization of patients with IgM pemphigoid. Methods: Skin biopsy specimens and sera from IgM-positive patients were assessed using histopathology, direct and indirect immunofluorescence microscopy, enzyme-linked immunosorbent assays, immunoblotting, cryosection assay, complement fixation test, and internalization assays. Results: Tissue-bound linear IgM deposits along the DEJ and circulating IgM autoantibodies against type XVII collagen (Col17) were detected. These circulating IgM autoantibodies showed no complement activating or blister inducing capacity, but the ability of Col17 internalization ex vivo. Limitations: Limited number of patients. Conclusion: This study provides further evidence for the role of IgM autoantibodies in pemphigoid disease and highlights Col17 as a target antigen in IgM pemphigoid.