Mortality of bullous pemphigoid: an evaluation of 223 patients and comparison with the mortality in the general population in the United States.

Background: There are large discrepancies in reported mortality for bullous pemphigoid (BP).

Objective: We sought to determine the mortality of a large cohort of patients with BP and compare this with age-matched control subjects.

Methods: Data were collected on 223 patients with a new diagnosis of BP between 1998 and 2003 through our cutaneous immunofluorescence laboratory databases. The mortality of patients with BP was compared with that of age-matched control subjects in the general US population.

Results: The 1-, 2-, and 5-year mortality was 0.23 (95% confidence interval=0.18, 0.29), 0.37 (95% confidence interval=0.31, 0.44), and 0.50 (95% confidence interval=0.42, 0.57), respectively. However, relative to age-matched control subjects, no difference in expected mortality was detected.

Limitations: This was a retrospective cohort analysis.

Conclusions: Mortality of patients with BP is more likely related to advanced age and associated medical conditions than to disease-specific factors.