Autoimmune bullous diseases

Abstract

Bullous autoimmune dermatoses are the result of an autoantibody-driven immune response against structural proteins of the skin. The cause of autoantibody formation is largely unknown. Three disease groups of bullous autoimmune dermatoses are distinguished, which differ clearly in pathogenesis and clinical picture, although they all typically manifest with inflammatory blisters of the skin: pemphigus and pemphigoid diseases, as well as dermatitis herpetiformis (Duhring's disease). In pemphigus diseases, autoantibodies are formed against desmosomal adhesion proteins that mediate epidermal cell-cell contact. This leads to intraepidermal blister formation. In contrast, in pemphigoid diseases the blisters are subepidermal and are caused by autoantibodies against hemidesmosomal structural proteins or anchoring fibrils of the dermo-epidermal junction zone with subsequent recruitment of granulocytes into the skin.