Epidemiological study of pemphigus vulgaris in the southwest of Iran: a 15-year retrospective study and a review of literature

Pemphigus vulgaris (PV) is a life-threatening autoimmune disease that affects the skin and mucosa. In most regions, little data is available on its epidemiology. This study aimed to present an overview of the worldwide epidemiology of pemphigus and evaluate the epidemiological features of PV in the southwest of Iran. In this study, data including sex, age, time of disease onset, phenotype of the disease, geographic location of birth, geographic location of residence, level of education, and occupation of PV patients from 2004 to 2019 were collected from the records existing in the Oral Medicine Department of Shiraz Dental School and Shahid Faghihi Hospital, Shiraz, Iran. In total, 249 PV patients with a mean age of 42.72 ± 13.3 years were included. The female to male ratio was 1.74:1. The annual incidence of PV in Fars province was 2.7 per million people. In this region, the most frequent phenotype was the mucocutaneous phenotype in 204 patients (81.9%), followed by the mucosal dominant phenotype in 40 (16.1%) and the cutaneous phenotype in 5 (2%). Regarding the level of education, 43.4% had less than a high school diploma, 24.9% had a high school diploma, 17.3% had an associate diploma or higher, and 14.5 % were illiterate. Moreover, 55.8% were housewives, 21.7% were self-employed, 19.3 % were employed, and 3.2% were jobless. The findings indicate that PV is a rare disease in the southwest of Iran compared with other regions. According to previous studies in this region, the incidence has decreased since 2005.