Prognosis

Most patients go into clinical remission with appropriate treatment. Nevertheless, mortality is considerable among older patients, with an estimated death rate between 6% and 41% in the first year.[50][51]

The reported mortality of bullous pemphigoid 1 year after initiating therapy is much greater in Europe (19% to 41%) than in the US (6% to 12%).[50][51][8] The difference in outcome may be the result of differences in treatment, ethnic differences, age, study size, and patient selection bias.[25] In a survey of patients in one US university medical center, there was no difference between the mortality of 223 bullous pemphigoid patients and the general population.[52] However, cohort studies in the UK and France reported the risk of death was 2 to 6 times greater in bullous pemphigoid patients compared with the general population.[7][8]

In one multivariate analysis, age ≥86 years, poor general condition, female sex, and generalized disease were associated with increased risks of death at 6 months.[53] Results of a randomized controlled trial carried out in 20 dermatology departments in France indicated that only older age (P=0.02) and a low Karnofsky score (P <0.001) appeared to independently predict death.[25] [ Karnofsky Performance Status Score Opens in new window ]

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