Background and design: With the exception of some areas in Brazil, pemphigus is an exceptional disease. Several previous observations suggested that pemphigus foliaceus occurred more frequently than expected in Tunisia. To confirm these observations, a 15-month prospective survey was undertaken at the University Hospital of Sousse in Tunisia.
Results: During the survey, 23 patients with pemphigus were observed. Twenty of 23 cases were of pemphigus foliaceus that affected only young women. Seven patients had the clinical features of pemphigus herpetiformis. The estimated incidence of pemphigus foliaceus in the Sousse area was four new cases per million inhabitants per year, far above European or North American incidence but lower than the incidence in the most severely affected areas in Brazil. The high prevalence of the herpetiform clinical variant, the young age, the feminine exclusivity, and the absence of familial cases differentiated this Tunisian pemphigus both from pemphigus foliaceus observed in Europe and North America and from the Brazilian fogo selvagem. No etiologic factor was identified.
Conclusions: This study suggests the existence in Tunisia (and possibly in all North Africa) of an endemic form of pemphigus foliaceus restricted to young women.