A case of antiepiligrin cicatricial pemphigoid successfully treated by plasmapheresis

We report the case of a 73-year-old Japanese woman suffering from antiepili grin (laminin 5) cicatricial pemphigoid (CP) with typical clinical and immu nopathological features. Histologically, the lesional mucous membrane showe d a subepidermal blister formation. When indirect immunofluorescence techni ques with skin split by 1 M NaCl as the substrate were used, the patient's serum reacted only to the dermal side. Immunoprecipitation studies demonstr ated that the patient's serum contained IgG autoantibodies directed against a set of polypeptides that corresponded to epiligrin (laminin 5). After co rticosteroids and immunosuppressive agents had been administered systemical ly, the patient's autoantibody titer decreased and the cutaneous and mucosa l blister formations were suppressed. However, the ocular lesions persisted in spite of these therapeutic regimens. After combining these treatments w ith double-filtration plasmapheresis, the ocular lesions improved and showe d almost no progression. Plasmapheresis may thus present a new option for t he treatment of CP. Copyright (C) 2000 S. Karger AG. Basel.