Epidemiological data of 290 pemphigus vulgaris patients: a 29-year retrospective study.

Abstract

Pemphigus vulgaris (PV), an autoimmune blistering disease involving the skin and mucosa, is traditionally considered to be prevalent among Jews, particularly those of Ashkenazi origin. Israel, where the Ashkenazi and non-Ashkenazi Jewish population live alongside a large Arab minority, is a particularly interesting place for epidemiological studies of PV. To characterise the epidemiological and clinical parameters of PV patients from a single tertiary medical centre in Israel. Data was retrieved retrospectively from the medical records of newly diagnosed PV patients referred to the Sheba Medical Center between 1980 and 2009. A total of 290 PV patients were diagnosed during the study period. The mean age at diagnosis was 49.7 years (range: 10-92 years) and a female predominance was identified (1.54:1; p<0.001). Among the Jewish patients, the ratio of Ashkenazi to non-Ashkenazi was 1.23:1, which was not statistically significant in comparison to the ratio of the general Jewish population in Israel (p=0.289). We describe the comorbidities found among the patients. Disease severity at diagnosis was not found to be related to the epidemiological parameters examined. Studies from different countries reveal variations in the clinical and epidemiological characteristics of the disease. The epidemiology of PV in Israel, a Middle-Eastern country with a Western lifestyle and a diverse ethnic population, shows some characteristics that represent an "admixture" between European and Middle-Eastern or Asian countries. The associated comorbidities of PV emphasize the need for dermatologists to keep a high index of suspicion and actively evaluate patients to determine their presence.