Epidemiology

Pemphigus is rare and, in the US, occurs with an overall incidence estimated as 4.2 new cases per million per year.[6] Most patients who develop pemphigus vulgaris, the most frequently occurring form of pemphigus, are aged >40 years.[5][7][8]​​​ The peak incidence is 50 to 60 years of age. However, the range is broad, with many cases reported in the paediatric age group. 

Pemphigus is more common in certain ethnic groups, including Ashkenazi Jewish and Mediterranean populations.[9][10]​​ In a Jewish population in the US (predominantly Ashkenazi Jews), the reported pemphigus incidence was 32 cases per million per year.[6]

One meta-analysis reported a pooled global incidence of pemphigus vulgaris of 2.83 per million person-years.[11]​ The incidence rate was highest in Asia (3.87 per million person-years; 11 studies) and lowest in Africa (1.38 per million person-years; 2 studies).[11]​

The incidence was not significantly different between men and women or between studies conducted after 2010 versus before 1990.[11]

In France, the mean annual crude incidence is estimated at 2 cases per million per year.[12]​​ In Korea, a nationwide population-based cohort study reported an incidence of 2.06 cases per million person-years for pemphigus vulgaris, and 1.14 cases per million person-years for pemphigus foliaceus.[13] In Finland, the incidence of pemphigus increased from 0.75 to 2.80 cases per million persons during the period 1985 to 2017.[14]

The overall US point prevalence for pemphigus is estimated to be 5.2 per 100,000 adults.[9] Globally, the reported prevalence of pemphigus vulgaris ranges from 30 per 100,000 people per year in Iran to 0.38 per 100,000 people per year in Bulgaria.[8]

There is less data on pemphigus foliaceous, but the reported incidence ranges from 0.5 to 3.9 per year per million people.[8] The reported prevalence of pemphigus foliaceous ranges from 0.6 to 10.01 per million people.[8]

Endemic PF (fogo selvagem) is found in high prevalence in isolated areas of Brazil. The prevalence of endemic PF was about 3% in certain areas of Brazil in the 1980s and 1990s but has since declined, linked to improvements in living conditions.​​[10][15]

Paraneoplastic pemphigus is rare and data are limited. Its estimated incidence is less than one new case per million people per year.​[16]

Use of this content is subject to our disclaimer