Department of Dermatology, Mayo Clinic, Rochester, Minnesota.
Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota.
J Am Acad Dermatol. 2014 Jul;71(1):92-9. doi: 10.1016/j.jaad.2014.02.030. Epub 2014 Apr 3.
Bullous pemphigoid (BP) is an autoimmune blistering disease that is associated with increased mortality.
We sought to determine the incidence and mortality of patients with BP.
A total of 87 residents of Olmsted County, Minnesota, were identified who had their first lifetime diagnosis of BP from January 1960 through December 2009. Incidence and mortality were compared with age- and sex-matched control patients from the same geographic area.
The adjusted incidence of BP was 2.4 per 100,000 person-years (95% confidence interval, 1.9-2.9). Incidence of BP increased significantly with age (P < .001) and over time (P = .034). Trend tests indicate increased diagnosis of localized disease (P = .006) may be a contributing factor. Survival observed in the incident BP cohort was significantly poorer than expected (P < .001). Survival was not different among patients with multisite versus localized disease (P = .90).
Retrospective study design and study population from a small geographic area are limitations.
Incidence of BP in the United States is comparable with that found in Europe and Asia. The mortality of BP is lower in the United States than Europe, but higher than previous estimates.
大疱性类天疱疮（BP）是一种自身免疫性水疱病，与死亡率增加有关。
我们旨在确定 BP 患者的发病率和死亡率。
总共确定了 87 名明尼苏达州奥姆斯特德县的居民，他们在 1960 年 1 月至 2009 年 12 月期间首次被诊断为 BP。将发病率和死亡率与来自同一地理区域的年龄和性别匹配的对照患者进行比较。
调整后的 BP 发病率为 2.4/100,000 人年（95%置信区间，1.9-2.9）。BP 的发病率随年龄（P<0.001）和时间（P=0.034）显著增加。趋势检验表明，局部疾病的诊断增加（P=0.006）可能是一个促成因素。在 BP 发病队列中观察到的生存情况明显差于预期（P<0.001）。多部位与局限性疾病患者的生存无差异（P=0.90）。
回顾性研究设计和小地理区域的研究人群是局限性的。
美国的 BP 发病率与欧洲和亚洲相当。BP 的死亡率在美国低于欧洲，但高于以前的估计。
