Intraepithelial autoimmune bullous dermatoses disease activity assessment and therapy

Abstract

Intraepithelial autoimmune blistering dermatoses are a rare group of skin disorders characterized by disruptions of inter-keratinocyte connections within the epidermis through the action of autoantibodies. The second article in this continuing medical education series presents validated disease activity scoring systems, serologic parameters of disease, treatments, and clinical trials for pemphigus and its subtypes.

Keywords: IVIG; Pemphigus Disease Activity Index; azathioprine; dapsone; intraepithelial autoimmune bullous dermatoses; mycophenolate mofetil; paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome; pemphigus erythematosus; pemphigus foliaceus; pemphigus herpetiformis; pemphigus vulgaris; rituximab.