Morini J P, Jomaa B, Gorgi Y, Saguem M H, Nouira R, Roujeau J C, Revuz J
Dermatology Service, Henri Mondor Hospital, Créteil, France.
Arch Dermatol. 1993 Jan;129(1):69-73. doi: 10.1001/archderm.129.1.69.
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.
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.
This study suggests the existence in Tunisia (and possibly in all North Africa) of an endemic form of pemphigus foliaceus restricted to young women.
除巴西的某些地区外，天疱疮是一种罕见疾病。先前的多项观察表明，落叶型天疱疮在突尼斯的发病率高于预期。为证实这些观察结果，在突尼斯苏塞大学医院进行了为期15个月的前瞻性调查。
在调查期间，共观察到23例天疱疮患者。23例中有20例为落叶型天疱疮，仅累及年轻女性。7例患者具有疱疹样天疱疮的临床特征。苏塞地区落叶型天疱疮的估计发病率为每年每百万居民中有4例新发病例，远高于欧洲或北美的发病率，但低于巴西受影响最严重地区的发病率。疱疹样临床变异型的高患病率、患者年轻、仅女性发病以及无家族病例，使得这种突尼斯天疱疮既不同于在欧洲和北美观察到的落叶型天疱疮，也不同于巴西的类天疱疮。未发现病因。
本研究提示，在突尼斯（可能在整个北非）存在一种局限于年轻女性的地方性落叶型天疱疮。
