Proteoglycans in albo-papuloid lesions of the Pasini form of dominant dystrophic epidermolysis bullosa.

Epidermolysis bullosa is a group of inherited blistering diseases classified into three main sub-groups on the basis of the level of cleavage within the skin. In dominant dystrophic epidermolysis bullosa, characterized by cleavage below the basal lamina, two variants can be distinguished by the presence (Pasini form) or absence (Cockayne-Touraine form) of albo-papuloid lesions. The present study was undertaken to investigate the glycosaminoglycan chains of proteoglycans in the albo-papuloid lesions of a patient with the Pasini form, using histochemical and immunohistochemical methods. Histological examination revealed no dermo-epidermal separation. In the dermis, the papillary and subpapillary layers were slightly homogeneous, and exhibited a strong affinity towards alcian blue, which was abolished by treatment with chondroitinase ABC or in the presence of MgCl2 0.3M, but was resistant to digestion with streptomyces hyaluronidase. The papillary and subpapillary layers were intensely stained with a monoclonal antibody against small size proteoglycan with dermatan sulfate. These results suggest the presence of degraded dermatan sulfate proteoglycan in the papillary and subpapillary dermis of albo-papuloid lesions in the Pasini form of dystrophic epidermolysis bullosa.