Immunohistological localization of three basement membrane components in various forms of epidermolysis bullosa

Abstract

The skin biopsies of eight epidermolysis bullosa (EB) patients, representing epidermolytic, junctional and dermolytic forms of the disease were studied immunohistochemically using antibodies against collagen Types IV and V and a proteoglycan. All these molecules arc either basement membrane components or closely associated substances. In two types of EB simplex (subtype of the epidermolytic form) the splicing took place above the basement membrane, whereas the staining with all three antibodies remained localized to the floor of the blister The herpetiform variant of EB simplex proved to be junctional, i.e. the separation occurred within the lamina lucida. One patient clinically classified as belonging to the junctional EB group, was found to have the epidermolytic form of the disease In this case all antibodies were localized only on the floor of the blister. In the patients with the dermolytic form of EB, all the antibodies stained the roof of the blister. The immunofluorescence techniques are rapid and easy to perform and are therefore proposed as useful for routine clinical diagnosis.