Recurrent Vogt-Koyanagi-Harada disease presenting with diffuse orbital inflammation

PURPOSE: To report diffuse orbital inflammation as a manifestation of recurrent inflammation in a patient with Vogt-Koyanagi-Harada (VKH) disease. OBSERVATIONS: 20-year-old African American male, who was previously diagnosed with VKH, presented with right eye pain, swelling, and binocular double vision. He had run out of methotrexate while on steroid taper. Neuroimaging was consistent with diffuse orbital inflammation with myositis. He was started on intravenous steroids and then transitioned to oral steroids, with complete resolution of his symptoms. CONCLUSIONS AND IMPORTANCE: Central nervous system involvement as a manifestation of VKH has been previously reported, however, there have been no reports of orbital inflammatory syndrome resulting from VKH. Thus, in the appropriate clinical context, orbital signs may be recognized as features of recurrent VKH.