Dermatology Hospital of Jiangxi Province, Jiangxi Provincial Clinical Research Center for Skin Diseases, Candidate Branch of National Clinical Research Center for Skin Diseases, Dermatology Institute of Jiangxi Province, The Affiliated Dermatology Hospital of Nanchang University, Nanchang, China.
Key Laboratory of Modern Preparation of Traditional Chinese Medicine, Ministry of Education, Jiangxi University of Traditional Chinese Medicine, Nanchang, China.
Front Immunol. 2023 Mar 9;14:1149119. doi: 10.3389/fimmu.2023.1149119. eCollection 2023.
Mucous membrane pemphigoid (MMP) is a type of subepithelial autoimmune bullous disease, affecting various mucosae, occasionally with skin lesions. Both diagnosis and treatment of MMP are difficult. Although multiple autoantigens have been identified for MMP, the pathogenesis of MMP is still unclear. In this study, we presented a female MMP case with extensive oral mucosal lesions and skin lesions, particularly on the extremities. IgG and IgA autoantibodies against multiple autoantigens including BP180, laminin 332, integrinα6β4 and desmoglein 3, and IgM autoantibodies against BP180 were identified during the disease course. Compared with IgG autoantibodies, the levels of IgA autoantibodies against various autoantigens decreased more significantly with improvement of clinical features after the initiation of treatments. Our findings indicated the importance of comprehensive autoantibody screening for different immunoglobulin types and autoantigens at multiple time points for the precise diagnosis of various autoimmune bullous diseases, and the significant involvement of IgA autoantibodies into the pathogenesis of MMP.
黏膜性类天疱疮（Mucous membrane pemphigoid，MMP）是一种亚上皮自身免疫性大疱病，影响多种黏膜，偶尔伴有皮肤损伤。MMP 的诊断和治疗均较为困难。尽管已经确定了 MMP 的多种自身抗原，但 MMP 的发病机制仍不清楚。本研究报道了一例 MMP 女性患者，其口腔黏膜广泛受累，皮肤损伤，尤其是四肢。在疾病过程中检测到针对多种自身抗原（包括 BP180、层粘连蛋白 332、整合素α6β4 和桥粒芯糖蛋白 3）的 IgG 和 IgA 自身抗体，以及针对 BP180 的 IgM 自身抗体。与 IgG 自身抗体相比，在开始治疗后，随着临床特征的改善，针对各种自身抗原的 IgA 自身抗体的水平下降更为显著。我们的研究结果表明，在多个时间点对不同免疫球蛋白类型和自身抗原进行全面的自身抗体筛查对于各种自身免疫性大疱病的精确诊断非常重要，并且 IgA 自身抗体在 MMP 的发病机制中具有重要作用。
