Annual incidence and mortality of bullous pemphigoid in the Grampian Region of North-east Scotland

Background Data on the annual incidence of bullous pemphigoid (BP) in the U.K. are scarce. Objective To estimate the annual incidence of BP in Grampian Region (North-east Scotland) and to assess the causes of mortality in this cohort of patients. Methods Details were obtained of all patients with a diagnosis of BP recorded in the database of the Pathology Department, Aberdeen Royal Infirmary between January 1991 and December 2001. Community Health Index population data were obtained from the Grampian Health Board and the annual incidence and age- and sex-specific incidence were calculated. Mortality data were obtained from the Patient Administration System and causes of death obtained from the Office of the Registrar for Births and Deaths for Scotland. Results Eighty-three patients met criteria for diagnosis of BP. The annual incidence of BP in Grampian region was estimated to be 14 cases per million per year. There was a clear and marked rise in the incidence in patients over the age of 80 years. Forty-eight per cent of patients with BP died within 2 years of diagnosis. The all-cause age-standardized mortality ratio was 576%. When compared with cause-specific mortality in the Grampian population over 60 years of age, respiratory disease accounted for a higher than expected number of deaths in our cohort of patients with BP (odds ratio 5.3, 95% confidence interval 3.0-9.4). Conclusion North-east Scotland appears to have a relatively high incidence of BP when compared with incidence rates in continental Europe. The mortality rate in patients with BP is considerable, especially within the first 2 years of diagnosis.