Mortality Rate in Bullous Pemphigoid: A Retrospective Monocentric Cohort Study

Abstract

Background: Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease and is associated with an increased mortality. The end points of our study were to evaluate the mortality rate in a retrospective cohort of BP patients followed up to 5 years after the diagnosis and to determine prognostics factors. Methods: All new cases of BP diagnosed between 1990 and 2003 in the University Hospital of Geneva were retrospectively collected. 60 patients were included, 47 (88.6%) of whom were treated with a combination of corticosteroids and chlorambucil. Results: The 1-year, 2-year and 5-year probabilities of death were 26.7, 37.1 and 60.8%, respectively. Old age, dementia and use of chlorambucil at initial doses of 6 mg/day, but not at lower doses, were associated with poor prognosis in multivariate analysis. Conclusion: Our study confirms that BP is associated with a high mortality. The observed mortality rates are however higher than those of previous studies, which is probably related to the inclusion of more debilitated patients.

Mortality of patients with bullous pemphigoid in Korea

“…These rates are lower than those reported in European studies. [18][19][20] However, the mean age of the patients in our study was 69.15 years (SD, 15.70), which was approximately 10 years younger than that of previous European studies. Considering the younger age of our patient population, our results are consistent with previous data from Switzerland.…”

“…[17][18][19] However, Continued J AM ACAD DERMATOL VOLUME 71, NUMBER 4 in our cohort, the age of $ 70 years could be a risk factor for poor prognosis in BP patients. Interestingly, other groups have reported diabetes to be a poor prognostic factor for overall survival 15,18 ; however, a history of diabetes showed an increased risk of 1-year mortality but not overall mortality in our study. Diagnosis delay was also an independent predictive factor of 1-year mortality but not overall mortality.…”

“…13,[15][16][17][18][19][21][22][23] However, some of the data from these studies are based on 1-year mortality rate and others are based on overall mortality. Therefore, we analyzed prognostic factors associated with both 1-year and overall survival.…”

“…15,17,18,22,23 However, some of these studies have classified dementia, cerebrovascular disease, and psychosis collectively as neurologic disease. Therefore, in this study we analyzed There are some limitations to our study.…”

Prognostic factors for mortality in patients with bullous pemphigoid: a meta-analysis

“…However, the asymmetry in funnel plot may also simply be the play of chance, since only ten studies were included, or true heterogeneity existed because cut-off age varied greatly. Of note, the two outlier investigations by Roujeau et al [29] and Cortés et al [5] were small studies with less than 100 participants. Nevertheless, the above finding indicates that younger age at the diagnosis of BP does not necessarily denote a lower risk of mortality, and physicians should be more cautious about patients’ general health condition and comorbidity profile.…”