The treatment of pemphigus vulgaris. Experience with 48 patients seen over an 11-year period

We present a retrospective analysis of 48 patients with pemphigus vulgaris (PV) who were seen between 1978 and 1988. They were divided into three treatment groups: 25 (group A) received 40-100 mg of oral prednisone daily; eight (group B) received > 100 mg of prednisone daily; 15 (group C) received 40 mg of oral prednisone every other day and azathioprine 100 mg daily. A second immunosuppressive agent was subsequently added to the treatment regimen of three patients in group A and eight patients in group B. By 1989, 10% of the patients had been able to discontinue all therapy, and were in complete remission. Sixty-five per cent of patients were on maintenance therapy, but in clinical remission. Twenty-five per cent of the patients had died [eight in group A (31%) and four in group B (50%)] either as a consequence of the disease or its treatment. None of the patients in group C had died. Most of the deaths occurred during the first 2-3 months of therapy. Morbidity and mortality were related to the severity of the disease, to the maximum dose of prednisone required to induce remission, and to the presence of other diseases. Patients needing a total of 5 g or more of prednisone to induce a remission during the acute stage had a high mortality rate.