Epidermolysis bullosa acquisita: Ultrastructural and immunological studies

Abstract

Four patients with epidermolysis bullosa acquisita were investigated using immunofluorescence, routine electron microscopic and immunoelectron microscopic techniques. Immunofluorescence studies demonstrated linear immunoglobulin and complement deposition along the dermal-epidermal junction. These findings are similar to those seen in skin of patients with bullous pemphigoid. Immunoelectron microscopic studies demonstrated that the immunoreactants were localized below the subbasal lamina-anchoring fibril zone of the basement membrane, thereby clearly distinguishing the immunopathology of epidermolysis bullosa acquisita from that seen in bullous pemphigoid. Indirect immunoelectron microscopic findings suggest that epidermal basal cells of affected patients may secrete the dermal substances to which the antibodies bind.