A Narrative Review of Pemphigoid Diseases: Bridging Associations, Comorbidities, and Management.

Abstract

Pemphigoid diseases are a group of rare, chronic autoimmune blistering disorders (AIBD) characterized by subepidermal blister formation due to autoantibody-mediated targeting of structural components of the basement membrane zone (BMZ). This narrative review provides an updated overview of four key subtypes: bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA bullous dermatosis (LABD), and epidermolysis bullosa acquisita (EBA) with specific emphasis on disease associations that dermatologists should be aware of for comprehensive patient care. BP, the most common subepidermal blistering disorder, is frequently associated with neurological and cardiovascular comorbidities, while MMP primarily affects mucosal surfaces, often leading to significant scarring and functional impairment and can be associated with malignancy in certain subtypes. LABD and EBA are less common but can be linked with systemic autoimmune conditions such as inflammatory bowel disease (IBD). Shared challenges across these subtypes include difficulties in diagnosis due to overlapping clinical and histopathologic features, treatment-related complications from prolonged corticosteroid use, and the substantial impact on patients' quality of life. Advances in therapeutic strategies, including biologic agents such as rituximab and dupilumab, offer promising alternatives to traditional treatments, yet access to new targeted therapies remains a significant barrier. This review emphasizes the need for large-scale studies to better understand disease associations, the identification of biomarkers for personalized therapy, improved treatment options, and the implementation of multidisciplinary care approaches.