大疱性类天疱疮合并神经系统疾病22例临床分析

目的总结大疱性类天疱疮(bullous pemphigoid,BP)合并神经系统疾病的特点,探讨二者间可能存在的病理机制联系.方法收集2001年3月至2005年3月在我院门诊就诊及住院的BP患者的临床资料,对其中22例合并有神经系统疾病患者的临床资料进行分析.结果BP平均发病年龄为(74.4±9.3)岁;BP诊断前病程(4.2±4.4)个月.神经系统疾病平均发病年龄(74.4±9.3)岁,包括痴呆、脑血管病13例(脑血栓、脑出血、脑梗死等)和多系统萎缩1例.BP均发生在神经系统疾病起病之后.治疗后随访0.5～48个月,6例失访,4例死亡.结论大疱性类天疱疮合并神经系统疾病的情况并不少见.二者合并出现的机制尚不清楚,免疫交叉反应或年龄相关的某些变性退化过程可能参与激发大疱性类天疱疮.

A clinical study on the association of bullous pemphigoid and neurological diseases 22cases report

Objective To study the clinical features of the patients who were diagnosed bullous pemphigoids (BP) and neurological diseases concomitantly and to investigate the pathologic relationship between the two identities. Methods To collect the related data of the BP cases that were diagnosed in Peking Union Medical College Hospital during the 4-year period (2001-2005). A retrospective study was made to those patients who were associated with neurological disorders. Results 109 patients of BP were collected, 22 had neurological diseases, including dementia,cerebral stroke (cerebral thrombosis, cerebral hemorrhage and cerebral infarction) and multiple system atrophy. In all patients, the onset of bullous pemphigoid was subsequent to neurological disorders. Conclusions The association between BP and neurological diseases is not rare. The pathologic mechanisms of this concomitancy are still not clear. It is proposed that immunological cross-reactions or neurodegenerative processes in relation to ageing trigger the development of BP. These studies might explain why BP is so strongly linked with the geriatric.