Bullous pemphigoid is associated with high mortality and considerable burden of disease in the elderly population, according to study data published in the British Journal of Dermatology reports.

The incidence and prevalence of bullous pemphigoid in England from 1998 to 2017 were examined using retrospective longitudinal electronic health record data from the Clinical Practice Research Datalink and linked Hospital Episode Statistics. The incidence and annual point prevalence were calculated per 100,000 person-years and per 100,000 people, respectively. Sociodemographic factors, incidence rate ratios, mortality rates, and burden of disease were all examined in multivariable adjusted analyses.

From 1998 to 2017 in England, there were 2658 incident cases of bullous pemphigoid (median age at presentation, 81 years). During the observational period, the pooled incidence of bullous pemphigoid was 7.63 (95% CI, 7.35-7.93)/100,000 person-years. The incidence rate increased with increasing age, with elderly men experiencing the greatest increase (P for interaction <.001).

Incidence increased by 1.2% (rate ratio [RR], 1.012; 95% CI, 1.005-1.020; P =.002) each year. The estimated annual increase in incidence was 0.9% (RR, 1.009; 95% CI, 1.002-1.017; P =.016) in the analysis adjusted for age and sex. During the almost 20-year period, prevalence of bullous pemphigoid almost doubled. Prevalence reached 47.99 (95% CI, 43.09-53.46)/100,000 people in 2017 from 26.82 (95% CI, 23.83-30.19)/100,000 people in 1998.


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In addition, prevalence reached 141.24 (95% CI, 125.55-158.87)/100,000 people aged >60 years. All-cause mortality risk was significantly higher at 2 years after diagnosis (hazard ratio [HR], 2.96; 95% CI, 2.68-3.26). The risk for all-cause mortality remained raised every year thereafter (HR, 1.54; 95% CI, 1.36-1.74).

Limitations of the study included its retrospective nature and the reliance on clinical codes for identifying cases of bullous pemphigoid.

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Although the risk for mortality associated with bullous pemphigoid has remained relatively stable during the years, the investigators of this study emphasize that “improved management, a better understanding of the etiology, and examination of the causes of death in bullous pemphigoid are still required.”

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Reference

Persson MSM, Harman KE, Vinogradova Y, et al. Incidence, prevalence, and mortality of bullous pemphigoid in England 1998-2017: A population-based cohort study. Br J Dermatol. 2020 Mar 9. doi: 10.1111/bjd.19022