title: Mortality of bullous pemphigoid in Switzerland: a prospective study.
abstract: BACKGROUND
  Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and has been associated with a significant morbidity and an increased mortality rate.
  
  
  OBJECTIVE
  Our aims were: (i) to evaluate the mortality rate of BP in Swiss patients during the first 3 years after the diagnosis of BP, as the primary endpoint; and (ii) to compare this mortality rate with that of the general Swiss population and determine prognostics factors, as secondary endpoints.
  
  
  METHODS
  All new cases of BP diagnosed in Switzerland between 1 January 2001 and 31 December 2002 were collected prospectively by means of a standardized data collection form. After collection of the data, the treating physicians were asked to specify the course and evolution of the disease. In total, 115 patients were included in our study.
  
  
  RESULTS
  The 1-, 2- and 3-year probability of death were 20·9%, 28·0% and 38·8%, respectively. The mortality rate of patients with BP was three times higher than that of age- and sex-matched members of the general Swiss population. Besides age, the presence of neurological diseases at diagnosis was associated with increased mortality in multivariate analysis.
  
  
  CONCLUSIONS
  This is the first prospective study analysing the mortality rate of BP in an entire country. The calculated mortalities are in the lower range of those reported in previous European studies. However, in line with the latter, our findings confirm a high case-fatality rate for BP, with an increased 1-year mortality rate compared with the expected mortality rate for age- and sex-adjusted general population.
authors.affiliation: Service de Dermatologie, Hôpitaux Universitaires de Genève, 1211 Geneva 14, Switzerland. Begonia.Cortes@hcuge.ch
authors.forename: B
authors.initials: B
authors.lastname: Cortés
authors.forename: G
authors.initials: G
authors.lastname: Marazza
authors.forename: L
authors.initials: L
authors.lastname: Naldi
authors.forename: C
authors.initials: C
authors.lastname: Combescure
authors.forename: L
authors.initials: L
authors.lastname: Borradori
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002423:Cause of Death; D002648:Child; D015897:Comorbidity; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D010391:Pemphigoid, Bullous / Q000401:mortality*; D011379:Prognosis; D011446:Prospective Studies; D013557:Switzerland / Q000453:epidemiology; D055815:Young Adult