BACKGROUND: Endemic pemphigus foliaceus (EPF) or fogo selvagem (FS) is an endemic autoimmune disease, characterized by flaccid bullae induced by IgG(4) subclass antibodies. The authors demonstrate the epidemiological and clinical status of patients who have been followed at the University Hospital of Brasilia (HUB) for more than 15 years. METHODS: One hundred and ninety-six patients with FS took part in the project. In setting up this study a historical descriptive cohort of patients was put together. In order to collect data, the authors used a questionnaire where the patient indicated the sex, age at the onset, occupation, origin, clinical status, including scalp compromise, evolution, cofactors influencing clinical worsening and the treatment compliance. In order to minimize loss to follow up, the authors used the statistical method of incidence density (patients/years). RESULTS: The disease occurred in 58.4% of the young patients in the 11-30-year age bracket, and 52% came from urban areas. These patients included students and teachers. Localized disease predominated as compared with the generalized presentation of this condition. Fifty-nine percent of the patients evolved to the recurrent form. Those patients with the evolutional form, in remission for more than 1 year (94%), had been followed for more than 5 years. Even patients with the less active forms of the disease had scalp lesions. Thus scalp lesions are not an indicator of bad prognosis. CONCLUSIONS: In the present study, the disease affected patients from a higher sociocultural class than previously described. Furthermore, in contrast to other reports, a substantial number of the patients lived in urban areas, although often spending some time in a rural setting for leisure or professional activities. This study suggests that the longer the follow up, the higher the likelihood that the disease would progress to a more controlled clinical presentation. Scalp lesions were not related to adverse prognosis. Sun, heat and infections act as triggering factors for the immunological imbalance, worsening the disease.
BACKGROUND: Endemic pemphigus foliaceus (EPF) or fogo selvagem (FS) is an endemic autoimmune disease, characterized by flaccid bullae induced by IgG(4) subclass antibodies. The authors demonstrate the epidemiological and clinical status of patients who have been followed at the University Hospital of Brasilia (HUB) for more than 15 years. METHODS: One hundred and ninety-six patients with FS took part in the project. In setting up this study a historical descriptive cohort of patients was put together. In order to collect data, the authors used a questionnaire where the patient indicated the sex, age at the onset, occupation, origin, clinical status, including scalp compromise, evolution, cofactors influencing clinical worsening and the treatment compliance. In order to minimize loss to follow up, the authors used the statistical method of incidence density (patients/years). RESULTS: The disease occurred in 58.4% of the young patients in the 11-30-year age bracket, and 52% came from urban areas. These patients included students and teachers. Localized disease predominated as compared with the generalized presentation of this condition. Fifty-nine percent of the patients evolved to the recurrent form. Those patients with the evolutional form, in remission for more than 1 year (94%), had been followed for more than 5 years. Even patients with the less active forms of the disease had scalp lesions. Thus scalp lesions are not an indicator of bad prognosis. CONCLUSIONS: In the present study, the disease affected patients from a higher sociocultural class than previously described. Furthermore, in contrast to other reports, a substantial number of the patients lived in urban areas, although often spending some time in a rural setting for leisure or professional activities. This study suggests that the longer the follow up, the higher the likelihood that the disease would progress to a more controlled clinical presentation. Scalp lesions were not related to adverse prognosis. Sun, heat and infections act as triggering factors for the immunological imbalance, worsening the disease.