To evaluate survival and factors predicting death in bullous pemphigoid.Design
Retrospective analysis of cohort.Setting
Three referral centers (university hospitals).Patients
Among 237 patients recruited between January 1, 1985, and December 31, 1992, 20 were excluded because of doubtful diagnosis. The 217 remaining patients were 79 +/- 11 years old (mean +/- SD); 120 were women and 97 were men; and 79% had been treated with oral corticosteroids, 40 to 90 mg/d.Interventions
Missing information on follow-up was minimized by letters and/or telephone calls to patients, families, nursing homes, and physicians.Main Outcome Measures
Actuarial survival curve, compared with the expected curve as derived from census data, and evaluation of prognostic factors by comparing initial characteristics between patients alive at 6 months and these who died before that point.Results
Survival curve demonstrated an early increased mortality: 17% at 3 months and 31% at 6 months, mainly from sepsis and cardiovascular diseases. Of the factors related to bullous pemphigoid activity (duration; pruritus; and number and extent of blisters, eosinophilia, and serum antibodies) only generalized pemphigoid was predictive of death in comparison with localized forms. In multivariate analysis, age of 86 years or more (relative risk, 7.1; 95% confidence interval [CI], 2.0-25.4; P < or= to.01), poor general condition (relative risk, 8.2; 95% CI, 3.0-22.4; P < or= to.001), female sex (relative risk, 2.4; 95% CI, 1.1-5.4; P < or= to.05), and generalized disease (relative risk, 4.4; 95% CI, 1.4-13.7; P < or= to.01) were associated with increased risks of death at 6 months.Conclusion
In this series, generalized bullous pemphigoid had a poor prognosis especially in older patients and those in poor general condition.