Acquired skin disease of hemidesmosomes.

摘要: Abstract The hemidesmosome is a membrane-associated supramolecular dermal–epidermal complex linking the cytoskeleton of basal keratinocyte to structures within papillary dermis. Different components this have been identified as autoantigens in autoimmune bullous skin diseases. Some characterized at molecular level. Little known, however, about factors that initiate production autoantibodies. By histopathology, acquired diseases hemidesmosomes show subepidermal blisters and by direct immunofluorescence, linear deposits IgG, C3 or IgA junction. Bullous pemphigoid (BP) most common disease hemidesmosomes. Two proteins, BP180 BP230, primary targets autoantibodies BP. In addition, pemphigoid/herpes gestationis, lichen planus pemphigoides, cicatricial are an immune response BP180. Laminin 5 another well-characterized anchoring filament-lamina densa component Patients with laminin clinical phenotype pemphigoid. Other reveal plectin-like protein, β4 subunit α6β4 integrin, uncein not yet 168 kDa protein. Recently, 105 200 proteins lower lamina lucida reported. association these still needs be demonstrated. Finally, fibrils associate complex. major structural type VII collagen, autoantigen epidermolysis bullosa acquisita.