فهرست نشریات علمی دسترسی آزاد ایران

Behçet’s Disease (BD) is a chronic, multisystem inflammatory disorder, typically characterized by recurrent oral and genital ulcers, uveitis, and skin lesions. However, atypical presentations—particularly with isolated neurological or ocular involvement—can delay diagnosis. We present a rare case of Behçet's disease in an 18-year-old male who presented with retrobulbar neuritis and neurological symptoms in the absence of active oral ulcers. The patient initially presented with unilateral retrobulbar neuritis, persistent headaches, dizziness, and cutaneous manifestations including erythema nodosum and papulonecrotic lesions. Remarkably, no oral ulcers were observed during active disease. The Pathergy test was initially positive but turned negative after initiating treatment. A comprehensive evaluation including cranial computed tomography, laboratory markers, and HLA-B51 testing was performed. Differential diagnoses such as multiple sclerosis and neurosarcoidosis were carefully excluded. The diagnosis of atypical Neuro-BD was established based on clinical criteria and exclusion of mimickers. Treatment with high-dose corticosteroids, azathioprine, colchicine, and acetazolamide was initiated. The absence of classic mucosal findings and the occurrence of rare symptoms such as retrobulbar neuritis pose significant diagnostic challenges, emphasizing the diversity of manifestations of BD. This case highlights the importance of considering atypical BD in patients with unexplained neurological and ocular symptoms. Retrobulbar neuritis, although rare in BD, may be the initial manifestation. The dynamic change in Pathergy test reactivity during treatment further underscores the complexity of disease monitoring. Early recognition and tailored therapy are essential for favorable outcomes.