Pemphigus vulgaris in older adults

Pemphigus vulgaris (PV) affects primarily middle-aged persons in North America. Conventional therapy suggests use of high doses of systemic corticosteroids and immunosuppressive agents. We have observed a group of older patients with PV and determined their clinical characteristics, treatment, and clinical course.

Eighteen patients with PV, in whom the onset of the disease was at age 65 or older, were observed and followed at the New England Baptist Hospital in Boston, Massachusetts, between 1985 and 1992. We determined sex and race distribution, disease severity, clinical presentation, autoantibody levels, treatment, response to treatment, and clinical course in these patients.

Men and women were affected equally. All the patients were white, and 50% were Jewish. Extensive disease was observed in only 27.8%. The disease was localized in 44.4%; in eight patients (44.4%), the use of sublesional corticosteroid (SLS) injection alone produced satisfactory clinical response. In patients with severe disease, the maximum dose of 60 mg/day of prednisone was sufficient. Patients with moderate disease responded to 20 to 40 mg/day of prednisone. Immunosuppressive agents were used in only four patients. The patients were followed for 3 to 15 years (mean 5.2 years). None of them died of the disease or the complications of therapy. At the time we reported this experience, most of the patients were in clinical remission without any therapy.

Our observations suggest that prognosis for older patients with PV is favorable. In several older patients, PV was controlled by SLS or lower doses of systemic corticosteroids. Systemic agents should be used cautiously and judiciously, and supportive measures should be offered frequently and aggressively.