Influence of treatment on the clinical course of pemphigus vulgaris.

Abstract

Pemphigus vulgaris (PV) is a potentially fatal autoimmune blistering disease. Our purpose was to summarize reported results of treatment of PV and suggest a basis for future studies. This retrospective review applies objective criteria to 77 studies published during the last half century. It includes only patients older than 18 years of age with idiopathic PV and excludes patients with drug-induced PV. Systemic corticosteroids significantly (p=0.001) reduced the mortality rate associated with PV compared with no treatment in the presteriod era. Adjuvants used with steroids significantly (p=0.001) reduced the mortality rate compared with the steroid era. The mortality rate of Jewish patients is significantly (p=0.001) higher than for non-Jewish patients. The outcome of PV is not influenced by the site of the initial lesion. Although the retrospective nature of this review limits its validity, we conclude prednisone with an adjuvant is the preferred treatment, and methotrexate should be avoided.