Yang Liuyiyi, Wang Yulu, Zuo Yagang
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, 9 Dongdan 3rd Alley, Beijing, 100730, China.
Department of Dermatology, Xiajin Country People's Hospital, Dezhou, Shandong, China.
Arch Dermatol Res. 2025 Jan 24;317(1):303. doi: 10.1007/s00403-024-03760-0.
Bullous pemphigoid (BP) is a debilitating autoimmune skin blistering disease, characterized by the deposition of specific autoantibodies at the dermal-epidermal junction. This leads to an inflammatory cascade involving the activation of complement proteins, mast cell degranulation, immune cell recruitment, and the release of proteases by granulocytes. While several cytokines and signaling pathways have been implicated in the pathogenesis of BP, the precise mechanism behind autoantibody production remains unclear. A variety of factors, including natural aging, genetic polymorphisms, microbiota, medications, vaccinations, and infection, may contribute to disease onset. Recent evidence also suggests that both vaccination against severe acute respiratory syndrome coronavirus-2 and infection with severe acute respiratory syndrome coronavirus-2 may also play a role in BP's development. This review aims to elucidate the mechanism underlying the production of autoantibodies in BP, address gaps in understanding disease progression, and explore opportunities for improving diagnosis and prognosis to enhance patient care.
大疱性类天疱疮（BP）是一种使人衰弱的自身免疫性皮肤水疱病，其特征是特定自身抗体在真皮-表皮交界处沉积。这会引发一系列炎症反应，包括补体蛋白的激活、肥大细胞脱颗粒、免疫细胞募集以及粒细胞释放蛋白酶。虽然几种细胞因子和信号通路与BP的发病机制有关，但自身抗体产生背后的确切机制仍不清楚。多种因素，包括自然衰老、基因多态性、微生物群、药物、疫苗接种和感染，都可能导致疾病发作。最近的证据还表明，接种严重急性呼吸综合征冠状病毒2疫苗和感染严重急性呼吸综合征冠状病毒2也可能在BP的发展中起作用。这篇综述旨在阐明BP中自身抗体产生的潜在机制，填补对疾病进展理解的空白，并探索改善诊断和预后以提高患者护理水平的机会。
