BACKGROUND: Bullous pemphigoid is associated with poorly understood dramatically increased early mortality rates. OBJECTIVES: To assess the incidence of acute events predisposing to early mortality. METHODS: Computerized medical records from the Health Improvement Network, a large population-based U.K. general practice database, were used to conduct a cohort analysis. Outcome measures were incidence rates of myocardial infarction, pulmonary embolism, pneumonia and sepsis compared with a matched control population. RESULTS: People with bullous pemphigoid were three times as likely to develop pneumonia, adjusted rate ratio 2.94 [95% confidence interval (CI) 2.01-4.31] or pulmonary embolism, adjusted rate ratio 3.12 (95% CI 1.37-7.12) compared with matched controls. No statistically significant increase was seen for myocardial infarction, adjusted rate ratio 1.24 (95% CI 0.66-2.33), or sepsis, adjusted rate ratio 2.02 (95% CI 0.78-5.21). CONCLUSIONS: The risk of pulmonary embolism and pneumonia is increased following a diagnosis of bullous pemphigoid. It may be possible to reduce associated mortality through considering prophylaxis with either antithromboembolic measures or antibiotic therapy and vaccination.