Pemphigus: Analysis of 1209 cases

Abstract

BACKGROUND: Pemphigus is a rare and chronic life-threatening disease. The clinical picture varies in reports from different regions of the world. OBJECTIVE: To define the clinical forms of pemphigus in a large cohort of patients. METHODS: Prospective analysis of 1209 patients diagnosed and followed at the Pemphigus Research Unit, Tehran University for Medical Sciences, from 1984 to 2003. RESULTS: The mean age at onset was 42 years with a female to male ratio of 1.5/1. The most frequent form was pemphigus vulgaris. In pemphigus vulgaris, patients' mucous membrane involvement alone was observed in 18%, skin involvement alone in 12%, and both in 70%. Pemphigus foliaceus was observed in 7% of the patients. Most complications were iatrogenic. CONCLUSION: In Iran, pemphigus vulgaris is the most frequent form of pemphigus. Females are more prone to the disease. The incidence of pemphigus in Tehran is approximately 1.6 per 100,000/year, and in Iran 1.0 per 100,000/year. The age of onset was lower than classically reported. Death occurred in 6.2% of the patients. In pemphigus vulgaris, the mucosal and skin form together had a worse prognosis than the other clinical forms.