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.