Conflicts of interest None declared.
Clinical and serological follow-up studies of endemic pemphigus foliaceus (fogo selvagem) in Western Parana, Brazil (2001–2002)
Article first published online: 3 MAY 2006
British Journal of Dermatology
Volume 155, Issue 2, pages 446–450, August 2006
How to Cite
Empinotti, J.C., Aoki, V., Filgueira, A., Sampaio, S.A.P., Rivitti, E.A., Sanches, J.A., Li, N., Hilario-Vargas, J., Diaz, L.A. and Cooperative Group on Fogo Selvagem Research (2006), Clinical and serological follow-up studies of endemic pemphigus foliaceus (fogo selvagem) in Western Parana, Brazil (2001–2002). British Journal of Dermatology, 155: 446–450. doi: 10.1111/j.1365-2133.2006.07302.x
- Issue published online: 3 MAY 2006
- Article first published online: 3 MAY 2006
- Accepted for publication 6 January 2006
- bullous diseases;
- desmoglein 1;
- fogo selvagem;
- pemphigus foliaceus
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.
Objectives 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.
Patients and 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.
Conclusion 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.