Pemphigus: analysis of 1209 cases.

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.