Bullous Pemphigoid Incidence
The incidence of bullous pemphigoid is 43 per million per year in the U.K., with lower rates of 7-13 per million per year in other parts of Europe, and the disease predominantly affects individuals over 70 years of age with equal sex distribution. 1
Global and Regional Incidence Data
The most comprehensive epidemiological data shows significant geographic variation:
- United Kingdom: The highest reported incidence at 43 per million per year represents the most recent and robust data from Western Europe 1
- Continental Europe: France and Germany report 6-7 cases per million per year, though earlier guidelines suggest this may be underestimated 1
- Global pooled estimates: A 2022 systematic review and meta-analysis found a cumulative incidence of 8.2 per million people (95% CI 4.8-13.7) and an incidence rate of 34.2 per million person-years (95% CI 19.2-60.7) 2
The substantial variation between U.K. data (43 per million) and other European countries (7-13 per million) likely reflects differences in case ascertainment methods and healthcare system surveillance rather than true geographic differences 1, 2.
Age-Specific Patterns
Bullous pemphigoid is predominantly a disease of the elderly, with the vast majority of cases occurring in individuals over 70 years of age 1. However, important caveats exist:
- The disease can affect younger adults and even children, though this is rare 1, 3
- Population-based studies show higher incidence rates, suggesting the burden in elderly populations may be underestimated 2
- The incidence appears to be increasing over time, likely due to the aging population and improved recognition 4, 5, 2
Sex Distribution
Bullous pemphigoid occurs equally in both sexes with no gender predilection 1. This equal distribution is consistent across all age groups and geographic regions studied.
Ethnic and Geographic Considerations
The available evidence shows regional variation but limited data on ethnic differences:
- European populations: Cumulative incidence of 10.3 per million people (95% CI 5.8-18.2) 2
- Asian populations: Lower cumulative incidence of 5.6 per million people (95% CI 3.5-9.0) 2
- The lower incidence in Asian populations may reflect true ethnic differences, healthcare access disparities, or differences in diagnostic practices 2
Clinical Implications for Incidence Trends
The incidence of bullous pemphigoid is rising and expected to continue increasing due to:
- Advancing age of the global population 4
- Improved diagnostic capabilities including ELISA testing 1
- Greater awareness among clinicians 5, 2
- Studies from more recent years consistently report higher incidence rates 2
Important Caveats
The true burden of bullous pemphigoid is likely underestimated for several reasons:
- High-quality studies with better case ascertainment report incidence of 13.3 per million people (95% CI 6.0-29.5), substantially higher than overall pooled estimates 2
- Population-based studies yield higher incidence than hospital-based studies 2
- Mild or atypical presentations may go undiagnosed 6
- The disease is no longer considered rare among the elderly population 5