Service de Dermatologie, Hôpitaux Universitaires de Genève, 1211 Geneva 14, Switzerland.
Br J Dermatol. 2011 Aug;165(2):368-74. doi: 10.1111/j.1365-2133.2011.10413.x.
Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and has been associated with a significant morbidity and an increased mortality rate.
Our aims were: (i) to evaluate the mortality rate of BP in Swiss patients during the first 3 years after the diagnosis of BP, as the primary endpoint; and (ii) to compare this mortality rate with that of the general Swiss population and determine prognostics factors, as secondary endpoints.
All new cases of BP diagnosed in Switzerland between 1 January 2001 and 31 December 2002 were collected prospectively by means of a standardized data collection form. After collection of the data, the treating physicians were asked to specify the course and evolution of the disease. In total, 115 patients were included in our study.
The 1-, 2- and 3-year probability of death were 20·9%, 28·0% and 38·8%, respectively. The mortality rate of patients with BP was three times higher than that of age- and sex-matched members of the general Swiss population. Besides age, the presence of neurological diseases at diagnosis was associated with increased mortality in multivariate analysis.
This is the first prospective study analysing the mortality rate of BP in an entire country. The calculated mortalities are in the lower range of those reported in previous European studies. However, in line with the latter, our findings confirm a high case-fatality rate for BP, with an increased 1-year mortality rate compared with the expected mortality rate for age- and sex-adjusted general population.
大疱性类天疱疮（BP）是皮肤最常见的自身免疫性表皮下水疱病，与较高的发病率和死亡率相关。
我们的目的是：（i）评估瑞士患者在诊断为 BP 后的前 3 年内的 BP 死亡率，作为主要终点；（ii）将该死亡率与瑞士一般人群的死亡率进行比较，并确定次要终点预后因素。
通过标准化数据收集表，前瞻性地收集了 2001 年 1 月 1 日至 2002 年 12 月 31 日期间在瑞士诊断的所有新 BP 病例。收集数据后，要求治疗医生指定疾病的过程和演变。共有 115 例患者纳入本研究。
1、2、3 年的死亡率分别为 20.9%、28.0%和 38.8%。BP 患者的死亡率是年龄和性别匹配的瑞士一般人群的三倍。除了年龄外，在多变量分析中，诊断时存在神经疾病与死亡率增加相关。
这是第一项在整个国家分析 BP 死亡率的前瞻性研究。计算出的死亡率处于以前欧洲研究报告的较低范围。然而，与后者一致，我们的研究结果证实了 BP 的高病死率，与年龄和性别调整后的一般人群的预期死亡率相比，1 年死亡率增加。
