title: Clinical and serological follow-up studies of endemic pemphigus foliaceus (fogo selvagem) in Western Parana, Brazil (2001-2002).
abstract: BACKGROUND
  Fogo selvagem (FS) has been described in several regions of Brazil, including the Western regions of the state of Parana. In 1990, Empinotti et al. reported case studies of 213 patients with FS that were collected from 1976 to 1988. The same author (J.C.E.) has observed that the frequency of cases in these regions of Parana has decreased.
  
  
  OBJECTIVE
  The purpose of this study was to clinically and serologically evaluate a small group of the patients originally reported in 1990 and compare data with a group of control individuals. These patients were treated at the onset of the disease with systemic steroids.
  
  
  METHODS
  Patients with FS, their unaffected relatives (n = 80) and genetically unrelated controls (n = 15) were identified during a field study from 1 May 2001 to 30 June 2002. Sera from nine patients with FS and six normal controls that were collected in the 1976-1988 evaluation were available for this study. The sera were tested by indirect immunofluorescence, enzyme-linked immunosorbent assay (ELISA) and immunoprecipitation using recombinant human desmoglein 1 (Dsg1).
  
  
  RESULTS
  Only 16 of the originally identified 213 patients with FS were found during the field studies. Thirteen of the 16 patients were in clinical and serological remission; 20% of normal controls (19 of 95) were positive in the Dsg1 ELISA. The majority of these subjects (17 of 19) were genetically related to FS patients. Six normal controls that were positive in the Dsg1 ELISA in the original survey were found to be negative or weakly positive in this evaluation.
  
  
  CONCLUSIONS
  The reduced frequency of positive serological markers of disease in patients and normal controls from Western Parana, as well as the absence of recurrent disease in previously identified patients, suggest that environmental antigenic stimulation of the population at risk may have decreased in recent years.
authors.affiliation: Department of Dermatology, Federal University of Rio de Janeiro School of Medicine, Rio de Janeiro, Brazil.
authors.forename: J C
authors.initials: JC
authors.lastname: Empinotti
authors.forename: V
authors.initials: V
authors.lastname: Aoki
authors.forename: A
authors.initials: A
authors.lastname: Filgueira
authors.forename: S A P
authors.initials: SA
authors.lastname: Sampaio
authors.forename: E A
authors.initials: EA
authors.lastname: Rivitti
authors.forename: J A
authors.initials: JA
authors.lastname: Sanches
authors.forename: N
authors.initials: N
authors.lastname: Li
authors.forename: J
authors.initials: J
authors.lastname: Hilario-Vargas
authors.forename: L A
authors.initials: LA
authors.lastname: Diaz
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D051183:Desmoglein 1 / Q000276:immunology*; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010375:Pedigree; D010392:Pemphigus / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D011379:Prognosis
chemical_list: D001323:Autoantibodies; D051183:Desmoglein 1