CICATRICIAL PEMPHIGOID - A PROBLEM TO TRE AT

Cicatricial pemphigoid is an autoimmune subepidermal blistering diseas e of the skin and mucous membranes. We report on eight patients in who m the clinical diagnosis was confirmed by direct immunofluorescence. T he patients' age averaged 69 years; seven were female and one male. In itial symptoms were erosions of the oral mucous membranes in four pati ents, skin blisters in three patients, and in one patient an ocular in volvement was the first manifestation of the disease. Indirect immunof luorescence on NaCl-split human skin revealed circulating IgG antibodi es binding to the roof of the artificial blister in three patients. Im munoblotting of epidermal and dermal extracts disclosed binding of IgG antibodies of one of these patients to an epidermal 230 kD protein, w hereas IgA-antibodies showed no specific binding. In four of five pati ents with a strong ocular involvement IgA deposits were found by direc t immunofluorescence. These studies were done on biopsies of perilesio nal skin or oral mucous membranes, and they were positive in one patie nt even before the first ocular lesions appeared. Therefore, finding o f IgA deposits by direct immunofluorescence may be taken as a prognost ic criterion allowing selection of the proper treatment. We treated si x patients with a dexamethasone-cyclophosphamide pulse therapy, while two patients received dapsone orally. The involvement of skin and oral mucous membrane responded well to both regimens, whereas the ocular l esions were progressive, except in one patient. On the basis of our ei ght cases, we discuss both options and limitations in the treatment of cicatricial pemphigoid and review new aspects of the pathogenesis of this disease.