High risk of death in elderly patients with extensive bullous pemphigoid

To evaluate survival and factors predicting death in bullous pemphigoid.

Retrospective analysis of cohort.

Three referral centers (university hospitals).

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.

Missing information on follow-up was minimized by letters and/or telephone calls to patients, families, nursing homes, and physicians.

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.

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 = .01), poor general condition (relative risk, 8.2; 95% CI, 3.0-22.4; P < or = .001), female sex (relative risk, 2.4; 95% CI, 1.1-5.4; P < or = .05), and generalized disease (relative risk, 4.4; 95% CI, 1.4-13.7; P < or = .01) were associated with increased risks of death at 6 months.

In this series, generalized bullous pemphigoid had a poor prognosis especially in older patients and those in poor general condition.