A 16-year-old girl had bilateral visual loss due to exudative retinopathy, weakness related to a diffuse myositis, and a macular erythematous rash. Skin and muscle biopsy was compatible with the diagnosis of dermatomyositis. Although the systemic manifestations promptly resolved on corticosteroid therapy, the visual loss was permanent. Presumed vasculitis resulted in diffuse retinal changes and optic nerve atrophy. Examination eight years later revealed an arteriovenous shunt in the peripheral retina.
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