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 ages >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 pediatric age group. The male-to-female ratio in the US is 1.

In a Jewish population in the US (predominantly Ashkenazi Jews), the reported pemphigus incidence was 32 cases per million per year.[6]

The overall US point prevalence for pemphigus is estimated to be 5.2 per 100,000 adults.[9]

Endemic PF (fogo selvagem) is found in high prevalence in isolated areas of Brazil. The prevalence of endemic PF has been best studied on an Amerindian reservation in Brazil and is estimated to be as high as 2.6%.[10]

No published data exist on the incidence of paraneoplastic pemphigus (PNP), but many cases have been reported in the literature since its original description in 1990.[11][12]

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