Estimates of Pemphigoid Prevalence in the United States Based on Age and Gender

Bullous pemphigoid
Bullous pemphigoid
Women 60 and older comprise the majority of cases of pemphigoid in the United States.

The occurrence of pemphigoid is rare in the United States, with women age ≥60 comprising the majority of cases. A gender- and age-adjusted population analysis was conducted in order to better comprehend the burden of the pemphigoid group of autoimmune blistering diseases in the United States, and the results were published in the Journal of the American Academy of Dermatology.

The investigators sought to assess the standardized overall and age-, gender-, and race-specific prevalence estimates of pemphigoid in the United States. The cross-sectional analysis used electronic health record data for a demographically heterogeneous population-based sample of >55 million individuals across all 4 US census regions.

The overall prevalence of pemphigoid was 0.012%, or 12 patients with the disease per 100,000 individuals. The prevalence of pemphigoid in people aged ≥60 was 0.038%;  37.7 per 100,000 patients. The prevalence of disease, which increased approximately 2-fold with each successive age-group, was highest in patients age ≥90 (123.6 per 100,000 patients; 95% CI, 115.2-132.5).

The adjusted prevalence of pemphigoid in women was 12.7 per 100,000 patients
(95% CI, 12.3-13.2), which was slightly higher than the prevalence in men (11.0 per 100,000 patients; 95% CI, 10.5-11.6). The adjusted prevalence was similar in African Americans (15.4 per 100,000 patients; 95% CI, 14.0-17.0) and Caucasians (13.5 per 100,000 patients; 95% CI, 13.0-13.9).

A major study limitation was that the analysis of electronic health data may have led to disease misclassification.

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The investigators concluded that the analysis of the pemphigoid burden in the United States offers an epidemiologic framework for the validation of potential disease associations, specifically neurologic conditions such as cerebrovascular disease, Parkinsons, dementia, multiple sclerosis, and epilepsy. This may offer additional insight into the risk factors, associated comorbidities, and potential treatment outcomes in patients with pemphigoid.


Wertenteil S, Garg A, Strunk A, Alloo A. Prevalence estimates for pemphigoid in the United States: a gender and age adjusted population analysis [published online August 27, 2018].    J Am Acad Dermatol. doi:10.1016/j.jaad.2018.08.030