Mortality of patients with bullous pemphigoid in Italy: a retrospective study of a monocentric experience

Background: Mortality of bullous pemphigoid (BP) is a variable parameter, depending especially on the area where the study was conducted. The 1-year mortality rate and the identification of clinic-therapeutic factors with potential prognostic value in patients with BP were evaluated in a cohort from a single referral center. Methods: We have reviewed medical records of patients with BP diagnosed at the Sant' Orsola-Malpighi Hospital in Bologna (Italy) between 2005 and 2019. Data collected included sex, age at diagnosis, laboratory findings, severity of disease, dosage of systemic treatments, age at death and comorbidities. Only patients who had at least 1 year of follow-up were included. Results: Eighty-five patients were included; the mortality rate was 7.1%. The mortality rate of patients treated with a moderate dosage of corticosteroids was no higher than that of patients treated with a low dosage. The log-rank test showed a statistically significant correlation between mortality and patients aged ≥85, BP230 positive, and CCIS≥4. Conclusions: Compared with similar studies, our results show a lower 1-year mortality rate. Advanced age at diagnosis and CCIS were confirmed as major independent factors associated with poor prognosis in BP. Administration of moderate dosage of oral corticosteroids seems to have an overall positive benefit-risk ratio, providing a good control of the disease and minimizing the risk of hospitalization, possible related complications and the mortality rate.