Autoimmune Bullous Diseases

Autoimmune bullous diseases are a heterogeneous group of skin diseases clinically characterized by erosions and/or blisters on the skin and mucous membranes. The autoantibodies targeting epidermal or subepidermal adhesion proteins lead to a loss in skin integrity, which manifests clinically as the formation of blisters. The site of blister formation is related to the clinical presentation—flaccid and fragile blisters in intraepidermal (pemphigus group) versus tense blisters in subepidermal autoimmune bullous diseases (pemphigoid group). Diagnosis is based on clinical manifestations, histopathology, immunofluorescence, and serological testing. Corticosteroids, mostly in conjunction with adjuvant immunosuppressants, are the standard treatment of pemphigus. Rituximab, an anti-CD20 monoclonal antibody, is a relatively new and effective drug in recalcitrant and resistant pemphigus. Topical or systemic corticosteroids are the mainstream bullous pemphigoid therapy. The use of immunosuppressants, doxycycline and dapsone, may be recommended in patients with contraindications to oral corticosteroids.