Service de Dermatologie, Hôpitaux Universitaires de Genève, Geneva, Switzerland. begonia.cortes @ hcuge.ch
Dermatology. 2012;225(4):320-5. doi: 10.1159/000345625. Epub 2012 Dec 21.
Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease and is associated with an increased mortality. The end points of our study were to evaluate the mortality rate in a retrospective cohort of BP patients followed up to 5 years after the diagnosis and to determine prognostics factors.
All new cases of BP diagnosed between 1990 and 2003 in the University Hospital of Geneva were retrospectively collected. 60 patients were included, 47 (88.6%) of whom were treated with a combination of corticosteroids and chlorambucil.
The 1-year, 2-year and 5-year probabilities of death were 26.7, 37.1 and 60.8%, respectively. Old age, dementia and use of chlorambucil at initial doses of 6 mg/day, but not at lower doses, were associated with poor prognosis in multivariate analysis.
Our study confirms that BP is associated with a high mortality. The observed mortality rates are however higher than those of previous studies, which is probably related to the inclusion of more debilitated patients.
大疱性类天疱疮（BP）是最常见的自身免疫性水疱性皮肤病，与死亡率增加有关。我们研究的终点是评估诊断后 5 年内回顾性 BP 患者队列的死亡率，并确定预后因素。
收集了 1990 年至 2003 年期间在日内瓦大学医院诊断的所有新的 BP 病例。共纳入 60 例患者，其中 47 例（88.6%）接受了皮质类固醇和苯丁酸氮芥联合治疗。
1 年、2 年和 5 年的死亡率分别为 26.7%、37.1%和 60.8%。多变量分析显示，老年、痴呆和初始剂量为 6mg/天的苯丁酸氮芥的使用（而不是低剂量）与预后不良相关。
我们的研究证实 BP 与高死亡率相关。然而，观察到的死亡率高于以前的研究，这可能与纳入更多虚弱患者有关。
