Yamaoka Toshifumi, Murota Hiroyuki, Katayama Ichiro
Department of Dermatology, Osaka University Graduate School of Medicine, Osaka, Japan.
J Dermatol. 2015 Mar;42(3):315-7. doi: 10.1111/1346-8138.12752. Epub 2015 Jan 9.
Behçet's disease (BD) is a relapsing systemic inflammatory disorder of unknown etiology involving systemic vasculitis. Vasculitis in BD results from the involvement of arteries, veins and blood vessels of all sizes, which leads to the three major manifestations of this condition: venous occlusion, arterial occlusion and aneurysm formation. Therefore, whole-body vascular involvement should always be considered in BD patients. Here, we describe the first appearance of an internal carotid-posterior communicating artery aneurysm, resulting in complete oculomotor nerve palsy in a BD patient. A 44-year-old Japanese man suffered from recurrent episodes of erythema nodosum that had presented on the lower extremities for the past 2 years. His condition was diagnosed as an incomplete type of BD based on relapsing oral and genital ulcers, skin eruptions, such as erythema nodosum and folliculitis, a positive pathergy test and systemic arthralgia. Ten years after his initial clinical presentation, he had manifestations of right-sided ptosis and cyclic dull pain in his right temporal region. Magnetic resonance imaging and angiography revealed a right internal carotid artery-posterior communicating artery aneurysm. Although oculomotor nerve palsy associated with internal carotid artery-posterior communicating artery aneurysm in a BD patient has not been reported previously, our report highlights the fact that this abnormal manifestation should be considered in those with vasculo-BD.
白塞病（BD）是一种病因不明的复发性全身性炎症性疾病，涉及系统性血管炎。BD中的血管炎是由动脉、静脉和各种大小的血管受累引起的，这导致了该疾病的三大主要表现：静脉闭塞、动脉闭塞和动脉瘤形成。因此，BD患者应始终考虑全身血管受累情况。在此，我们描述了一名BD患者首次出现颈内动脉-后交通动脉瘤，导致完全性动眼神经麻痹。一名44岁的日本男性在过去2年中反复出现下肢结节性红斑。根据复发性口腔溃疡和生殖器溃疡、皮肤疹（如结节性红斑和毛囊炎）、针刺试验阳性和全身性关节痛，他的病情被诊断为不完全型BD。在他首次临床表现10年后，他出现了右侧上睑下垂和右侧颞部周期性钝痛。磁共振成像和血管造影显示右侧颈内动脉-后交通动脉瘤。虽然此前尚未报道BD患者中与颈内动脉-后交通动脉瘤相关的动眼神经麻痹，但我们的报告强调了在血管性BD患者中应考虑这种异常表现这一事实。
