Geoepidemiologic considerations of auto-immune pemphigus

Sporadic and endemic forms of pemphigus foliaceus exist. Incidences of the various forms of pemphigus vary from country to country: pemphigus vulgaris is most common in Europe and the USA, whereas pemphigus foliaceus is more prevalent in Northern Africa, Turkey and Southern America. Endemic pemphigus foliaceus (also known as fogo selvagem and Brazilian pemphigus foliaceus) is a variant of the disease that is frequently diagnosed in some areas of Brazil and other underdeveloped areas of the world, including Tunisia and Colombia. Both genetic and environmental factors have been associated with the occurrence of auto-immune pemphigus and endemic pemphigus is very interesting from this point of view. Most of the patients with auto-immune pemphigus are aged 40–60 years at the onset of the disease. The incidence and prevalence of pemphigus are low. The demonstration of association of pemphigus vulgaris with HLA class II alleles is supported by various studies.