Epidermolysis bullosa acquisita: A clinical disorder of varied etiologies: Two cases and a review of immunologic and other reported findings

摘要: Epidermolysis bullosa acquisita (EBA) is an acquired mechanobullous disorder characterized by traumaticlly induced bullae in skin subjected to frictional injury, i.e., acral extensor surfaces or any point of sufficient mechanical distress. Two new patients with EBA are reported. Histopathologic findings were subepidermal sparse superficial perivascular mononuclear cell inflammatory infiltrates. The periodic acid-Schiff (PAS)—positive basement membrane zone (BMZ) was split between the floor and roof blisters most attached blister roof. Electron microscopy normal from one patient revealed a bandlike granular material within dermis immediately beneath basal lamina. Nonbranching filaments embedded material. second markedly diminished density anchoring fibrils but no Immunologic biopsies C′3, IgG, other immunoglobulins varying combinations both cases all reported cases. Comparisons direct immunofluorescent (IF) nine 223 bullous pemphigoid (BP) suggest that BMZ deposits IgG plus IgA and/or IgM occur more frequently than BP. Indirect IF studies patients' sera antibodies two samples our seven others. While BP characteristically yield positive BMZ, clinical, electron microscopic, some immunopathologic indicate diseases distinct.