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.
Highlights
Autoimmune bullous diseases are a group of chronic, blistering diseases of the skin, the most common of which is bullous pemphigoid (BP)
This nationwide cohort study has included all patients in Sweden diagnosed with BP by a dermatologist [International Classification of Diseases (ICD) code, ICD 10th revision (ICD-10): L12.0, L12.0A, L12.0B, L12.0W, L12.8 and L12.9] in the National Patient Register (NPR) for the first time between 2005 and 2012
The average annual incidence rate of BP for residents above 20 years of age was 6.6/100,000 (Fig. 1)
Summary
Autoimmune bullous diseases are a group of chronic, blistering diseases of the skin, the most common of which is bullous pemphigoid (BP). BP is associated with autoantibodies to different structures in the skin, resulting in skin losses and subepidermal blisters. Knowledge about the incidence of BP is still limited and most studies are hampered by a retrospective design and a small number of included BP patients (n = 27–140) [2,3,4, 10, 15, 18, 22, 23, 26, 29, 31,32,33]. A recent prospective study from Switzerland, including 140 BP patients, estimated the incidence to 1.2 cases/100,000 inhabitants/year [22].
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