RESUMEN
BACKGROUND: Pemphigus is a disease demonstrating an uneven geographic distribution. This is the first epidemiologic study of pemphigus in Iran. METHODS: Data were collected from the files of all newly registered patients with pemphigus at the sole dermatologic referral center for south-western Iran located in the city of Shiraz, during the period 1991-2000. The data were analyzed with regard to age, sex, subtypes of pemphigus, clinical presentation, and season of onset. RESULTS: The results showed that the mean incidence of the disease for the overall population in Shiraz was 0.67/10(5)/year. The male to female ratio was 1 : 1.33. The mean age at onset of the disease was 38 years (range, 12-93 years). The disease was most common in the third to fifth decades (62.1% of the overall patients). Pemphigus vulgaris was the most commonly encountered type, followed by pemphigus foliaceus (87.7% and 9%, respectively). The first manifestations of the illness most often occurred during winter (30.8% of the patients). CONCLUSIONS: The results reveal a high incidence of pemphigus vulgaris in south-western Iran compared with that encountered in other countries. Our patients also had a relatively young age at onset and a female predominance.