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. 2017 Nov;309(9):721-727.
doi: 10.1007/s00403-017-1778-4. Epub 2017 Sep 5.

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

Kristofer Thorslund  1 , Oliver Seifert  2   3 , Karin Nilzén  4 , Carina Grönhagen  5
Affiliations

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

Kristofer Thorslund et al. Arch Dermatol Res. 2017 Nov.

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.

Keywords: Bullous pemphigoid; Incidence; Population-based cohort study.

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Conflict of interest statement

Conflict of interest

The authors declare that they have no conflict of interest.

Funding

This study was funded by Karolinska Institutet, the Welander-Finsen Foundation and Sigurd and Elsa Goljes Foundation.

Ethical approval

The Regional Ethical Review Board in Stockholm, Sweden has approved this study.

Informed consent

For this type of study formal consent is not required.

Figures

Fig. 1
Fig. 1
Incident and prevalent cases of bullous pemphigoid (BP) 2001–2012 in Sweden. Number of cases diagnosed with BP in Sweden, 2001–2012, from the National Patient Register (NPR)—both inpatient and outpatient care. Patients regarded as incident cases were above 20 years of age and diagnosed with BP for the first time in 2005 (dark colored bars). To estimate the incidence rate for the past 8 years (2005–2012), we divided the average annual number of cases (n = 470) diagnosed with BP during 2005–2012 by the whole background population in Sweden (>20 years) during the corresponding years (n = 7,122,447). Incidence of BP in Sweden 2005–2012 for residents above 20 years of age according to data from the NPR = 470/7,122,447 = 6.6 cases/100,000 (95% CI 6.0–7.2)
Fig. 2
Fig. 2
a Age-specific incidence rate (IR) of BP from 2005 to 2012 in Sweden. Average age-specific IR of bullous pemphigoid (BP), adjusted for the Swedish population in 2005–2012 per 100,000 people. The number above the bars is the actual number of patients within each age group on which the incidence estimate is based. b Age- and gender-specific incidence rate (IR) of BP from 2005 to 2012 in Sweden. Average age- and gender-specific incidence rate of bullous pemphigoid and its subsets (adjusted to the Swedish population in 2005–2012 per 100,000 inhabitants). The number above the bars is the actual number of patients within each age and gender group on which the incidence estimate is based
See this image and copyright information in PMC

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