BACKGROUND Pemphigusis a bullous, rare and chronic autoimmune disease. There are two
major forms of pemphigus: vulgaris and foliaceus. Epidemiological data and
clinical outcome in patients diagnosed in the Brazilian Amazon states are
still rare. OBJECTIVES To study the occurrence of the disease during the study period and analyze
the epidemiological profile of patients, the most common subtype of
pemphigus, and the clinical evolution of patients. METHODS Retrospective analysis of medical records of hospitalized patients with
pemphigus foliaceus and pemphigus vulgaris in the period from 2003 to 2010
in Dermatology Service of Hospital Fundação Santa Casa de Misericórdia do
Pará, Belém, Northern Brazil. RESULTS We found a total of 20 cases of pemphigus during the study period, 8 of
which were of foliaceus pemphigus and 12 of vulgaris pemphigus. Pemphigus
foliaceus had the predominance of male patients (75%), showed satisfactory
clinical evolution, and was characterized by absence of pediatric cases.
Pemphigus vulgaris affected more women (66.7%), showed mean hospital stay of
1 to 3 months (50%), and there were three cases of death (25%). The
prescribed immunosuppressive drugs included prednisone with or without
combination of azathioprine and/or dapsone. Sepsis was associated with 100%
of the deaths. CONCLUSIONS The occurrence of the disease is rare, there are no familiar/endemic
outbreaks in the sample. Evolution is usually favorable, but secondary
infection is associated with worse prognosis. The choice of best drugs to
treat pemphigus remains controversial.