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. 2014 Jul;71(1):92-9.
doi: 10.1016/j.jaad.2014.02.030. Epub 2014 Apr 3.

Incidence of bullous pemphigoid and mortality of patients with bullous pemphigoid in Olmsted County, Minnesota, 1960 through 2009

Katherine E Brick  1 , Chad H Weaver  1 , Christine M Lohse  2 , Mark R Pittelkow  3 , Julia S Lehman  4 , Michael J Camilleri  4 , Mustafa Al-Hashimi  1 , Carilyn N Wieland  5
Affiliations

Incidence of bullous pemphigoid and mortality of patients with bullous pemphigoid in Olmsted County, Minnesota, 1960 through 2009

Katherine E Brick et al. J Am Acad Dermatol. 2014 Jul.

Abstract

Background: Bullous pemphigoid (BP) is an autoimmune blistering disease that is associated with increased mortality.

Objective: We sought to determine the incidence and mortality of patients with BP.

Methods: A total of 87 residents of Olmsted County, Minnesota, were identified who had their first lifetime diagnosis of BP from January 1960 through December 2009. Incidence and mortality were compared with age- and sex-matched control patients from the same geographic area.

Results: The adjusted incidence of BP was 2.4 per 100,000 person-years (95% confidence interval, 1.9-2.9). Incidence of BP increased significantly with age (P < .001) and over time (P = .034). Trend tests indicate increased diagnosis of localized disease (P = .006) may be a contributing factor. Survival observed in the incident BP cohort was significantly poorer than expected (P < .001). Survival was not different among patients with multisite versus localized disease (P = .90).

Limitations: Retrospective study design and study population from a small geographic area are limitations.

Conclusion: Incidence of BP in the United States is comparable with that found in Europe and Asia. The mortality of BP is lower in the United States than Europe, but higher than previous estimates.

Keywords: autoimmune blistering disorder; bullous pemphigoid; epidemiology; geriatric; incidence; mortality.

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Conflict of interest statement

Conflict of Interest: The authors state no conflict of interest.

Figures

Figure 1
Figure 1
Bullous Pemphigoid. Incidence of BP Showing Significant Increases With Age at Diagnosis.
Figure 2
Figure 2
Bullous Pemphigoid. Incidence of BP Showing Significant Increase Over Time.
Figure 3
Figure 3
Bullous Pemphigoid. Survival in the Incident BP Cohort With Significantly Poorer Than Expected Results.
See this image and copyright information in PMC

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