Immunotherapy for pemphigus and bullous pemphigoid

Abstract

Pemphigus and bullous pemphigoid (BP) are both autoimmune bullous diseases (AIBD), but their clinical features and treatment strategies are different. Pemphigus is characterized by intraepidermal blisters mediated by antibodies against desmosomes, while BP is characterized by subepidermal blisters mediated by antibodies against epidermal-dermal junctions. Traditional therapy for pemphigus and BP is mainly composed of systemic glucocorticoid and immunosuppressant. Rituximab targeting CD20 on B lymphocytes has been approved for first-line treatment for pemphigus and significantly improved clinical outcomes. Type 2 inflammation also plays a key role in the pathogenesis of BP. Biologic agents such as dupilumab targeting the interleukin-4 receptor have demonstrated promising efficacy in treating refractory BP patients. Additionally, various immune-targeted therapies have been investigated for their efficacy and safety in managing pemphigus and BP. This review summarizes recent advances and clinical applications of immunotherapy in these two major AIBDs.