J.V. Otten, T. Hashimoto, M. Hertl, A.S. Payne and C. Sitaru Pages 69 - 95 ( 27 )
Blister formation in skin and mucous membranes results from a loss of cell-cell or cell-matrix adhesion and is a common outcome of pathological events in a variety of conditions, including autoimmune and genetic diseases, viral and bacterial infections, or injury by physical and chemical factors. Autoantibodies against structural components maintaining cell-cell and cell-matrix adhesion induce tissue damage in autoimmune blistering diseases. Detection of these autoantibodies either tissue-bound or circulating in serum is essential to diagnose the autoimmune nature of disease. Various immunofluorescence methods as well as molecular immunoassays, including enzyme-linked immunosorbent assay and immunoblotting, belong to the modern diagnostic algorithms for these disorders. There is still a considerable need to increase awareness of the rare autoimmune blistering diseases, which often show a severe, chronic-relapsing course, among physicians and the public. This review article describes the immunopathological features of autoimmune bullous diseases and the molecular immunoassays currently available for their diagnosis and monitoring.
Autoantibodies, autoantigens, basement membrane, desmosome, ELISA, extracellular matrix, hemidesmosome, immunoassay, immunoblotting, immunofluorescence microscopy.
Department of Dermatology, University of Freiburg, Hauptstrasse 7, D-79104 Freiburg, Germany.