Sitaru C: Immunopathology and molecular diagnosis of autoimmune bullous diseases

Autoimmune bullous diseases are associated with autoimmunity against structural components maintaining cell–cell and cell–matrix adhesion in the skin and mucous membranes. Pemphigus diseases are characterized by autoanti-bodies against the intercellular junctions and intraepithelial blisters. In pemphigoid diseases and epidermolysis bul-losa acquisita, sub-epidermal blistering is associated with autoantibodies targeting proteins of the hemidesmosomal anchoring complex. The autoantigens in autoimmune blistering diseases have been extensively characterized over the past three decades. In general, the pathogenicity of autoantibodies, already suggested by clinical observations, has been conclusively demonstrated experimentally. Detection of tissue-bound and circulating serum autoantibod-ies and characterization of their molecular specificity is mandatory for the diagnosis of autoimmune blistering dis-eases.For this purpose, various immunofluorescence methods as well as immunoassays, including immunoblotting, enzyme-linked immunosorbent assay and immunoprecipitation have been developed. This review article describes the immunopathological features of autoimmune bullous diseases and the immunological and molecular tests used for their diagnosis and monitoring