Department of Ophthalmology, College of Medicine and Health Sciences, Sultan Qaboos University, Muscat, Oman.
Indian J Ophthalmol. 2011 May-Jun;59(3):240-1. doi: 10.4103/0301-4738.81048.
Behçet disease is an idiopathic, multisystem disorder characterized by recurrent episodes of orogenital ulceration and vasculitis of the veins and arteries of all calibers. Ocular involvement may affect the conjunctiva, sclera, uveal tract, vitreous, blood vessels, and retina. Many theories have pointed toward an autoimmune response behind its pathogenesis, which may be triggered by exposure to an infectious agent. Frosted branch angiitis is characterized by vascular inflammation, sheathing, retinal edema, and retinal hemorrhages. The disease may be idiopathic in a majority of the cases or may be associated with ocular and systemic pathology. Association between Behηet disease, Frosted branch angiitis, and neuroretinitis is not reported in literature. This uncommon combination reflects the varied systemic and ocular manifestations in Behçet disease, especially in patients who are not diagnosed and treated in time. We hereby report a case of bilateral frosted branch angiitis and neuroretinitis in a young male from Middle-east, suffering from Behçet disease.
白塞病是一种特发性、多系统疾病,其特征为反复发作的口-生殖器溃疡和所有口径的静脉和动脉血管炎。眼部受累可能影响结膜、巩膜、葡萄膜、玻璃体、血管和视网膜。许多理论都指向其发病机制背后的自身免疫反应,这可能是由暴露于感染因子引发的。白塞病性树枝状血管炎的特征是血管炎症、鞘膜、视网膜水肿和视网膜出血。在大多数情况下,该病可能是特发性的,也可能与眼部和全身病理有关。白塞病、白塞病性树枝状血管炎和神经视网膜炎之间的关联在文献中并未报道。这种不常见的组合反映了白塞病的各种系统和眼部表现,特别是在那些未及时诊断和治疗的患者中。我们在此报告一例来自中东的年轻男性双侧白塞病性树枝状血管炎和神经视网膜炎病例。