Bullous Pemphigus: A Comprehensive Review of a Complex Autoimmune Disease of the Skin and Mucous Membranes

Abstract

Pemphigus bullosus, also known as pemphigus vulgaris, is a rare and potentially serious autoimmune disease affecting the skin and mucous membranes, characterized by the formation of intraepidermal blisters due to the loss of cellular cohesion at desmosomal junctions. In this article, we present a comprehensive review of the entity, covering its epidemiology, etiopathogenesis, clinical manifestations, diagnosis and therapeutic options. We highlight the complexity and diagnostic challenges associated with bullous pemphigus, emphasizing the importance of careful clinicopathologic and immunofluorescent correlation for its proper identification. We also discuss in detail the multidisciplinary therapeutic approach required to achieve disease control, emphasizing the use of systemic immunosuppressants and biological therapies. In addition, we highlight the relevance of local therapy and care management, along with the need for close medical follow-up to assess response to treatment and minimize adverse effects. Finally, we highlight the importance of continued research in this complex clinical entity, in order to improve the understanding of its immunopathological mechanisms and to develop more specific and personalized therapies. In conclusion, this article provides a comprehensive and updated view of bullous pemphigus, with the aim of improving its clinical management and promoting a more efficient and effective therapeutic approach.