Autoantibodies to basement membrane collagen: epidermolysis bullosa simplex versus bullous pemphigoid.

As judged by passive hemagglutination and hemagglutination-inhibition assays, sera from six patients in one family with dominant epidermolysis bullosa contain clearly demonstrable titers of antibodies against the collagen C chain which is derived from basement membrane structures. Moreover, the circulating antibodies observed in these patients are apparently specific for the C chain as no titers were observed when using four additional well-characterized collagen chains in the indicated assays. In contrast to the results with sera from epidermolysis bullosa patients, sera from a series of age- and sex-matched healthy controls, as well as from a group of patients with bullous pemphigoid, did not contain antibodies to any of the test antigens. These results thus clearly differentiate the autoimmune response to basement membranes observed in bullous pemphigoid from that observed in epidermolysis bullosa simplex.