<p>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.</p>

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Associated factors related to production of autoantibodies and dermo-epidermal separation in bullous pemphigoid

  • Liuyiyi Yang,
  • Yulu Wang,
  • Yagang Zuo

摘要

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.