title: Evaluation of cases of pemphigus vulgaris and pemphigus foliaceus from a reference service in Pará state, Brazil.
abstract: 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.
  
  
  OBJECTIVE
  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.
authors: Universidade Federal do Pará, Belém, PA, Brazil.
authors: Carla Andréa Avelar
authors: CA
authors: Pires
authors: Universidade Federal do Pará, Belém, PA, Brazil.
authors: Viviane Brito
authors: VB
authors: Viana
authors: Universidade Federal do Pará, Belém, PA, Brazil.
authors: Fernando Costa
authors: FC
authors: Araújo
authors: Universidade Federal do Pará, Belém, PA, Brazil.
authors: Silvia Ferreira Rodrigues
authors: SF
authors: Müller
authors: Centro Universitário do Estado do Pará, Belém, PA, Brazil.
authors: Miguel Saraty de
authors: MS
authors: Oliveira
authors: Universidade do Estado do Pará, Belém, PA, Brazil.
authors: Francisca Regina Oliveira
authors: FR
authors: Carneiro
mesh_terms: D000328:Adult; D017677:Age Distribution; D001379:Azathioprine / Q000627:therapeutic use; D001938:Brazil / Q000453:epidemiology; D003430:Cross-Sectional Studies; D003622:Dapsone / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007902:Length of Stay; D008297:Male; D008499:Medical Records; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy / Q000453:epidemiology* / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D012189:Retrospective Studies; D018805:Sepsis / Q000150:complications; D017678:Sex Distribution; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D003622:Dapsone; D001379:Azathioprine; D011241:Prednisone