Incidence of bullous pemphigoid in Sweden 2005–2012: a nationwide population-based cohort study of 3761 patients

Abstract

Studies that report the incidence of bullous pemphigoid from validated nationwide population-based registries are rare. The aim of this study was to estimate the incidence of bullous pemphigoid in Sweden 2005–2012. A population-based open cohort study was designed including all patients diagnosed by a dermatologist with bullous pemphigoid (BP) in Sweden from 2005 to 2012 (n = 3761), identified from the National Patient Register (NPR). The diagnosis of bullous pemphigoid in the NPR was recently validated from medical records, histopathological and immunopathological data by our group in a previous study. The average annual incidence of bullous pemphigoid was 7.1/100,000 (95% CI 6.5–7.7). Female to male ratio was 1.2:1, mean age at diagnosis was 78.9 years. The age-specific incidence rate increased markedly after 80 years of age with an incidence peak between 90 and 99 years of age, 81.9/100,000 (95% CI 75.0–89.2). This large nationwide cohort study presents an adjusted incidence of BP of 7.1/100,000 (95% CI 6.5–7.7) in Sweden. The incidence of bullous pemphigoid is higher than expected and bullous pemphigoid is a common disease in the elderly population.

Psoriasis is associated with increased risk of bullous pemphigoid: A nationwide population‐based cohort study in Taiwan

“…A few epidemiological studies from the USA, Europe and Far East showed that the age‐specific incidence rate of BP increased markedly after 80 years of age . In our study, patients with coexisting BP and psoriasis were significantly younger than BP patients among the non‐exposed group.…”

“…A few epidemiological studies from the USA, Europe and Far East showed that the age-specific incidence rate of BP increased markedly after 80 years of age. 19,20 In our study, patients with coexisting BP and psoriasis were significantly younger than BP patients among the non-exposed group. Moreover, among psoriatic patients, BP occurred not only in old age, but was also found in those aged less than 60 years, an age group in which BP has not been reported to occur frequently.…”

“…In our study, patients with coexisting BP and psoriasis were significantly younger than BP patients among the non‐exposed group. Moreover, among psoriatic patients, BP occurred not only in old age, but was also found in those aged less than 60 years, an age group in which BP has not been reported to occur frequently . These findings may be attributed to the alteration of the antigenicity of the basement membrane in psoriatic patients at a younger age, stimulating anti‐basement membrane autoantibody formation and BP development earlier in life …”

“…Moreover, among psoriatic patients, BP occurred not only in old age, but was also found in those aged less than 60 years, an age group in which BP has not been reported to occur frequently. 19,20 These findings may be attributed to the alteration of the antigenicity of the basement membrane in psoriatic patients at a younger age, stimulating anti-basement membrane autoantibody formation and BP development earlier in life. 9 It is interesting to observe the relatively short duration (2.86 years) within which BP was diagnosed after psoriasis in our study.…”

Psychiatric comorbidity in individuals with bullous pemphigoid and all bullous disorders in the Danish national registers

“…The incidence of BP in Europe is estimated to be 2.5-42.8 cases/million/year [3][4][5][6][7][8][9], with incidence increasing over the past eight decades [10] and a nearly 300-fold increase in those over age 90 compared to those younger than 60 [4]. This rise may reflect increasing average life expectancy, increasing use of polypharmacy, and improved detection and diagnosis [5].…”