Purpose: To present the case of a young Turkish pregnant patient with bilateral, sight-threatening retinal ischemic vasculopathy and systemic signs suggestive of Behçet disease (oral ulceration, arthritis, and pseudofolliculitis).
Methods: Case report.
Results: Multiple areas of superficial, retinal white lesions and few hemorrhages related to occlusions of small retinal vessels were observed at presentation in the macular zone of both eyes. There was gradual improvement of retinal lesions after the administration of corticosteroids and immunosuppressive treatment.
Conclusion: We report the case of a young pregnant patient with bilateral, sight-threatening retinal ischemic vasculopathy and systemic signs of Behçet disease. Although the systemic signs were highly suggestive of Behçet disease, the ocular presentation was unusual for this multisystemic inflammatory disorder. The differential diagnosis included a number of causes of ischemic retinal vasculopathy, such as systemic vasculitis, antiphospholipid syndrome, Susac syndrome, Purtscher-like retinopathy, and a new variant of acute macular neuroretinopathy (paracentral, acute middle maculopathy), which are further discussed.