Epidermolysis bullosa acquisita

Abstract

Four patients with the clinical picture of epidermolysis bullosa acquisita were investigated. Biopsies were taken from the involved and uninvolved areas of the skin and the immunohistochemical and microscopic changes were studied. Direct immunofluorescence showed deposition of Ig[immunoglobulin]G and in a linear or notched pattern along the epidermal basement membrane in the involved and uninvolved skin. Ig[immunoglobulin]A (3/4), IgM (1/4), C4 (3/4) and properdin (3/4) could also be detected. Indirect immunofluorescence revealed the presence of circulating antibodies against inter alia the epithelial basement membrane zone in 1 patient. Routine EM showed that the blister was situated in the dermis leaving the basal lamina in the roof of the blister. With immunoelectron microscopy using peroxidase-labeled antibody the in vivo deposition of IgG was observed just beneath the basal lamina in the dermis of the perilesional and the uninvolved skin. Epidermolysis bullosa acquisita is a distinct entity in which autoimmune mechanisms might possibly play a role.