Original article
Mortality of patients with bullous pemphigoid in Korea

https://doi.org/10.1016/j.jaad.2014.05.006Get rights and content

Background

The reported mortality rates and prognoses of bullous pemphigoid (BP) vary among different countries. However, the mortality rate of BP has not been investigated in Korea.

Objective

We sought to evaluate the mortality rate of Korean patients with BP in comparison to that of an age-matched general population in Korea, and to identify prognostic factors affecting overall survival.

Methods

We conducted a retrospective analysis of 168 patients diagnosed with BP between 1993 and 2013 at Gangnam Severance Hospital in Seoul, Korea.

Results

The 1-, 2-, and 5-year mortality rates of BP patients were 19.46% (95% confidence interval [CI], 13.83-27.00), 29.13% (95% CI, 21.91-38.08), and 58.03% (95% CI, 47.04-69.45), respectively. The median age was 73 years (range, 6-99 years). The standardized mortality ratio ranged from 2.43 (95% CI, 0.12-11.96) to 9.56 (95% CI, 2.43-26.02), depending on the age group. In multivariate analysis, old age at the time of diagnosis, stroke, diabetes, and delayed diagnosis were associated with increased mortality.

Limitations

This study was conducted as a retrospective analysis and was based at a single institution.

Conclusion

The mortality rate of patients with BP is significantly higher than that for the general population.

Section snippets

Study population

We retrospectively reviewed the medical records of all patients with BP who were diagnosed at the Gangnam Severance Hospital in Seoul, Korea, between January 1993 and March 2013. The following criteria were used to diagnose BP: (1) clinical suspicion of BP because of characteristic features; (2) histopathology consistent with BP (ie, subepidermal blisters with infiltration of inflammatory cells, particularly eosinophils); (3) direct immunofluorescence studies revealing linear C3 or/and

Demographic data and clinical features of patients

A total of 168 patients with BP who met the diagnostic criteria were identified, and 84 (50%) patients were female. The median age at the time of diagnosis was 73 years, and the mean age was 69.15 years (standard deviation, 15.70). The age range at the time of diagnosis was 6 to 99 years, and the majority of patients (96%) were >40 years of age. There were 54 (32.14%) patients with mild BP and 45 (26.79%) patients with severe BP (Table I). The median duration of follow-up for those alive at the

Discussion

Regarding the mortality of BP patients, conflicting results have been reported from different countries. In our study of 168 Korean patients with BP, the 1-year mortality rate was 19%, the 2-year mortality rate was 29%, and the 5-year mortality rate was 58%. 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.

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    Funding sources: None.

    Conflicts of interest: None declared.

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