G. Hans et al., AN ACTIVE FOCUS OF HIGH PREVALENCE OF FOGO-SELVAGEM ON AN AMERINDIAN RESERVATION IN BRAZIL, Journal of investigative dermatology, 107(1), 1996, pp. 68-75
Fogo Selvagem (FS) is an autoimmune disease characterized by subcornea
l vesicles and antidesmoglein-1 autoantibodies. Previous epidemiologic
data have linked the onset of FS to exposure to an environmental anti
gen(s), This investigation describes a unique human settlement with an
extraordinarily high prevalence of FS, This community is made up of A
merindians belonging to the Terena tribe, which has settled on the Lim
ao Verde reservation in the state of Mate Grosso do Sul in Brazil, Twe
nty-six well-characterized FS cases have been identified within a tota
l population of 998, yielding a prevalence of 2.6%, Seventeen of the p
atients (65%) were males, and over 50% were older than 30 y of age. Th
e incidence of the disease shows temporal periodicity, i.e., years wit
h several cases of FS alternating with years with no cases. Over one-h
alf of the cases occurred in genetically related family members. Anoth
er Terena reservation, the Ipegue/Taunay, located 90 km west of the Li
mao Verde reservation, was also evaluated as a control group, This res
ervation, with a population of 2203, had no recorded cases of FS, Thus
, the Limao Verde reservation represents a new focus of FS in which th
e disease exhibits temporal, geographic, and familial clustering, Thes
e results suggest that the environmental antigen or antigens precipita
ting FS are endemic to the Limao Verde reservation, This reservation a
ppears to be an ideal population for carrying out sero-epidemiologic,
genetic, and environmental studies aimed at disclosing the etiology of
FS.