HIGH-RISK OF DEATH IN ELDERLY PATIENTS WITH EXTENSIVE BULLOUS PEMPHIGOID

Objective: To evaluate survival and factors predicting death in bullou s 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 exc luded because of doubtful diagnosis. The 217 remaining patients were 7 9+/-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. Interventio ns: Missing information on follow-up was minimized by letters and/or t elephone calls to patients, families, nursing homes, and physician. Ma in Outcome Measures: Actuarial survival curve, compared with the expec ted curve as derived from census data, and evaluation of prognostic fa ctors 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 facto rs related to bullous pemphigoid activity (duration; pruritus; and num ber and extent of blisters, eosinophilia, and serum antibodies) only g eneralized pemphigoid was predictive of death in comparison with local ized forms. In multivariate analysis, age of 86 years or more (relativ e risk, 7.1; 95% confidence interval [CI], 2.0-25.4; P less than or eq ual to.01), poor general condition (relative risk, 8.2; 95% CI, 3.0-22 .4; P less than or equal to.001), female sex (relative risk, 2.4; 95% CI, 1.1-5.4, P less than or equal to.05), and generalized disease (rel ative risk, 4.4; 95% CI, 1.4-13.7; P less than or equal to.01) were as sociated with increased risks of death at 6 months. Conclusion: In thi s series, generalized bullous pemphigoid had a poor prognosis especial ly in older patients and those in poor general condition.