Autoimmune bullous diseases: pathogenesis and clinical management

被引：0

作者：

Xun Feng ^{[1
]}

Huaping Zheng ^{[2
]}

Mi Wang ^{[1
]}

Yiyi Wang ^{[1
]}

Xingli Zhou ^{[1
]}

Xiwen Zhang ^{[1
]}

Jishu Li ^{[1
]}

Yue Xiao ^{[1
]}

Mintong Wei ^{[1
]}

Xiaoguang Li ^{[3
]}

Takashi Hashimoto ^{[4
]}

Jingyi Li ^{[1
]}

Wei Li ^{[1
]}

机构：

[1] West China Hospital,Department of Dermatology & Venerology, Rare Diseases Center

[2] Sichuan University,Department of Respiratory and Critical Care Medicine, Center for High Altitude Medicine, Institutes for Systems Genetics

[3] West China Hospital,Department of Dermatology, Graduate School of Medicine

[4] Sichuan University,undefined

[5] Daqing Oilfield General Hospital,undefined

[6] Osaka Metropolitan University,undefined

来源：

Molecular Biomedicine | / 6卷 / 1期

关键词：

Autoimmune bullous diseases; Autoantibody; Immunogenic domain; Pathogenesis; Targeted therapy; Inflammation;

D O I：

10.1186/s43556-025-00272-9

中图分类号：

学科分类号：

摘要：

Autoimmune bullous diseases (AIBDs) represent a heterogeneous group of immune-mediated disorders characterized by life-threatening blistering of the skin and mucous membranes. This Review synthesizes current understanding of AIBD pathogenesis, clinical phenotypes, diagnostic approaches, and therapeutic strategies, emphasizing recent advancements and translational opportunities. At the core of AIBDs is autoantibody-mediated disruption of structural proteins in the epidermis or basement membrane zone, particularly at desmosomal and hemidesmosomal junctions. Key subtypes, including pemphigus, paraneoplastic pemphigus, pemphigoid, and IgA-related diseases, are distinguished by their target antigens, clinical manifestations, and immunopathological profiles. Diagnostic workflows rely on direct immunofluorescence, and serological assays, yet subtype differentiation remains challenging due to overlapping features. Traditional therapies, such as systemic corticosteroids and immunosuppressants, have improved outcomes but are limited by toxicity. Recent breakthroughs highlight targeted interventions, including B-cell depletion with rituximab, cytokine modulation via dupilumab, and JAK inhibitors for inflammatory pathways. Innovative strategies like chimeric autoantibody receptor T-cell (CAART) therapy further address refractory cases by eliminating autoreactive B cells. Additionally, the Review underscores the emerging role of inflammation-driven mechanisms and the necessity of multidisciplinary care, given AIBDs’ associations with malignancies, autoimmune comorbidities. Despite progress, challenges persist in early diagnosis, personalized therapy optimization, and understanding antigen-specific immune responses. Future directions include refining diagnostic biomarkers, exploring novel targets, and developing precision medicine approaches.

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