Case Presentation: 11 y/o M presenting with 2 months of worsening headache and 2-3 days of rapidly progressive blurry vision. Patient initially presented to his school nurse after complaining in class of blurry vision. He was seen by a local optometrist and then transferred to the emergency department for further evaluation. Ophthalmologic evaluation in the emergency department showed pan-uveitis with bilateral retinal and optic nerve edema. Initial lab work showed normal CBC, CRP and ESR with positive ANA screen. Lumbar puncture was remarkable for an opening pressure of 35 cm H2O and acetazolamide was started. MRI brain and orbits showed small bilateral retinal hemorrhages and inflammatory changes consistent with uveitis. He was started on pulse dose steroids for concern for undifferentiated vasculitis. Extensive infectious workup including HSV and VZV were negative. Follow-up eye exam 2 days after therapy showed worsening disease with complete loss of peripheral vision along with pathognomonic findings of Behҫet’s disease (panuveitis, retinal vasculitis, and vascular occlusion). He was diagnosed with incomplete Behҫet’s disease and emergently started on infliximab and mycophenolate. On discharge patient had mild improvement of vision with plan to continue immunosuppressive therapy.
Discussion: Behҫet’s disease is an idiopathic inflammatory disease typically presenting with aphthous stomatitis, genital ulcerations and ocular disease. It is a clinical diagnosis with no specific laboratory findings. It affects more males than females with an onset age between 20 to 30 years. Though typically a disease of young adults, Behҫet’s may present in childhood. Males are more often affected by ocular and vasculitis symptoms.
Females more often present with genital ulcers and skin lesions. Diagnosis is typically made based on International Criteria for Behҫet’s Disease. Incomplete Behҫet’s refers to manifestations of the disease in only a few organ systems. Ocular involvement is an important cause of morbidity. While typical ocular involvement may progress with attack and then remission with full visual recovery, vision loss can occur, with posterior uveitis and retinal vasculitis being the main cause. Therefore, the American Uveitis Society recommends treatment the use of anti-tumor necrosis factor α as first line therapy for ocular involvement with Behҫet’s Disease.
Conclusions: Behҫet’s Disease is a rare but important cause of vision loss since ocular inflammatory disease is a leading cause of vision loss worldwide. Timely diagnosis is necessary as early treatment with anti-tumor necrosis factor α may halt disease progression and preserve visual capacity.