Subepidermal blistering disease with autoantibodies against a novel dermal200-kDa antigen

A number of autoimmune subepidermal blistering diseases are characterized b y the distinct autoantigens of the cutaneous basement membrane zone. Recent ly, a few cases with autoantibodies against a novel 200-kDa dermal protein have been reported. We collected nine cases of subepidermal blistering dise ase with IgG antibodies against this 200-kDa antigen. In this report, we de scribe the clinical and immunological appearances in these cases. Five case s showed bullous pemphigoid-like features, one case resembled dermatitis he rpetiformis, and another case showed mixed features of bullous pemphigoid a nd linear IgA bullous dermatosis. It was interesting to note that psoriasis coexisted in four cases. By indirect immunofluorescence on 1 M NaCl split skin, IgG antibodies from all sera reacted with the dermal side of the spli t. By immunoblot analysis, IgG antibodies recognized a 200-kDa protein of d ermal extract. IgG affinity-purified antibodies on the 200-kDa immunoblot m embrane stained the dermal side of 1 M NaCl split skin. Various examination s suggested that the 200-kDa antigen is not identical to any chains of lami nins-1. -5 or -6. This autoimmune subepidermal blistering disease against t he dermal 200-kDa protein may form a new distinct entity, which often assoc iates with psoriasis. (C) 2000 Elsevier Science Ireland Ltd. All rights res erved.