Hans-Filho G, dos Santos V, Katayama J H, Aoki V, Rivitti E A, Sampaio S A, Friedman H, Moraes J R, Moraes M E, Eaton D P, Lopez A L, Hoffman R G, Fairley J A, Giudice G J, Diaz L A
Departamento de Dermatologia, Universidade Federal de Mato Grosso do Sul, Brazil.
J Invest Dermatol. 1996 Jul;107(1):68-75. doi: 10.1111/1523-1747.ep12298213.
Fogo Selvagem (FS) is an autoimmune disease characterized by subcorneal vesicles and antidesmoglein-1 autoantibodies. Previous epidemiologic data have linked the onset of FS to exposure to an environmental antigen(s). This investigation describes a unique human settlement with an extraordinarily high prevalence of FS. This community is made up of Amerindians belonging to the Terena tribe, which has settled on the Limao Verde reservation in the state of Mato Grosso do Sul in Brazil. Twenty-six well-characterized FS cases have been identified within a total population of 998, yielding a prevalence of 2.6%. Seventeen of the patients (65 %) were males, and over 50% were older than 30 y of age. The incidence of the disease shows temporal periodicity, i.e., years with several cases of FS alternating with years with no cases. Over one-half of the cases occurred in genetically related family members. Another Terena reservation, the Ipegue/Taunay, located 90 km west of the Limao Verde reservation, was also evaluated as a control group. This reservation, with a population of 2203, had no recorded cases of FS. Thus, the Limao Verde reservation represents a new focus of FS in which the disease exhibits temporal, geographic, and familial clustering. These results suggest that the environmental antigen or antigens precipitating FS are endemic to the Limao Verde reservation. This reservation appears to be an ideal population for carrying out sero-epidemiologic, genetic, and environmental studies aimed at disclosing the etiology of FS.
灼口综合征（FS）是一种自身免疫性疾病，其特征为角膜下小水疱和抗桥粒芯糖蛋白-1自身抗体。先前的流行病学数据已将FS的发病与暴露于一种或多种环境抗原联系起来。本研究描述了一个FS患病率极高的独特人类聚居地。这个社区由属于泰雷纳部落的美洲印第安人组成，他们定居在巴西南马托格罗索州的利毛韦尔德保留地。在998人的总人口中已确诊26例特征明确的FS病例，患病率为2.6%。其中17名患者（65%）为男性，超过50%的患者年龄超过30岁。该病的发病率呈现出时间周期性，即FS病例数较多的年份与无病例的年份交替出现。超过一半的病例发生在有血缘关系的家庭成员中。另一个泰雷纳保留地，位于利毛韦尔德保留地以西90公里处的伊佩古/陶奈保留地，也作为对照组进行了评估。这个有2203人的保留地没有FS病例记录。因此，利毛韦尔德保留地代表了FS的一个新病灶，该病在其中呈现出时间、地理和家族聚集性。这些结果表明，引发FS的一种或多种环境抗原在利毛韦尔德保留地是地方性的。这个保留地似乎是开展旨在揭示FS病因的血清流行病学、遗传学和环境研究的理想人群。
