Pathophysiological characterization of autoimmune bullous skin diseases

SOUHRN DISERTAČNÍ PRÁCE V ANGLICKÉM JAZYCE Autoimmune bullous diseases are severe and chronic conditions, which involve skin and mucosal surface. The etiology is unknown. The specific antibodies against structural components of cellular adhesions molecules of epidermis, at the dermal-epidermal junctions or at the basement membrane zone, are characteristic. The connection between antibodies and targeted antigens leads to cell-cell or cell-matrix discontinuity, which develops into blister formation. Intraepidermal disruption is typical for pemphigus diseases, dermo-epidermal (sub- epidermal) blistering process is specific for pemphigoid diseases. Detection of specific autoantibodies either tissue-bound or circulating in serum is essential to diagnose autoimmune nature of autoimmune bullous disease. The specific antibodies for bullous pemphigoid against the hemidesmosomal antigens BP 180, BP 230 are seen, which connect the basal keratinocytes to basement membrane. The correct diagnosis is based on positivity of minimally 2 markers - histological proof, detecting of tissue- bound antibody Immunoglobulin G and C3 component of complement at the basement membrane zone by direct immunofluorescence, detection of circulating Immonoglobulin G by indirect imunofluorescence on the monkey or rabbit esophagus or..