Bullous pemphigoid (BP) is a chronic, acquired, autoimmune bullous disease characterized by subepidermal bullae. It is usually seen in the elderly but, rarely, may also be seen in children. Autoantibodies against hemidesmosomal proteins BP230 (BPAG1) and BP180 (BPAG2 or type XVII collagen) are blamed for the pathogenesis. Clinically, it is characterized by large, tense blisters. Blisters can occur on normal skin or erythematous base with a predilection of flexural aspects of the limbs, abdomen, groin and axillae. Mucous membrane involvement is seen in about 10–35% of the patients. For treatment, general maintenance of BP patients is the first step. Systemic steroids are the common treatment agents. But localized disease can be treated successfully with topical corticosteroids. The common immunosuppressive agents are azathioprine, mycophenolate mofetil, methotrexate, chlorambucil and less often cyclophosphamide. In a minority of resistant cases, intravenous immunoglobulins, plasma exchange, anti-CD20 immunotherapy (rituximab), leflunomide, chlorambucil and methotrexate may be effective.
Part of the book: Autoimmune Bullous Diseases