Despite being observed worldwide, the distribution of PV is ethnically and geographically unequal. Its incidence ranges from 0.76 new cases per million/year in Finland and 3.5 new cases per million/year in Japan to 16.1 cases per million/year in Jerusalem - worldwide, the incidence of this disease is highest in Ashkenazi Jews of Mediterranean origin.In most countries, PV is more frequent than PF - in France, for example, PV accounts for 73% of cases of pemphigus, and in Japan the ratio between PV and PF is 2:1. The exceptions are Finland, Brazil, and Tunisia; in the latter two, there are endemic foci of PF. In Brazil, endemic foci of PV are suspected in the central-west (Brasilia, DF) and southeast regions (Ribeirão Preto, SP). Studies present conflicting data regarding the evolution of PV incidence: while in Brazil and in the United Kingdom the incidence has increased in the last decade, in Israel a reduction was observed over the last 16 years. The male/female ratio ranges from 1:1.5 in Israel and Iran to 1:4 in Tunisia. PV may occur at any age, and disease onset is usually between 40 and 60 years of age. An increased frequency in the elderly and children has been observed. Interestingly, in some countries of the Middle East and Brazil, disease onset is earlier: a Brazilian study estimated that 17.7% of cases occur before the age of 30 years.