A 48-year-old female with no known medical history presented to the ophthalmology clinic for evaluation of blurred vision in both eyes without other concurrent symptoms. Detailed ophthalmologic examination revealed bullous, exudative subretinal fluid (Fig. 1A), patchy choroidal thickening, and peripheral pigmentary changes on a funduscopic exam, optical coherence tomography (OCT), and fluorescein angiogram (FA). A review of the systems and the rest of the physical examination were unremarkable. Acute phase reactants were normal and screening for diabetes mellitus, systemic lupus erythematosus, rheumatoid arthritis, Bechet’s disease, Susac and Cogan syndrome, sarcoidosis, and infectious etiologies was negative. A few months later, the patient noted decreased hearing and patchy depigmented areas on her skin primarily involving the face (Fig. 1B) and shoulders in addition to mild hair loss. An extensive audiogram demonstrated asymmetrical bilateral sensorineural hearing loss. The patient was diagnosed with Vogt-Koyanagi-Harada (VKH) disease and started on oral prednisone 1 mg/kg with a taper in addition to methotrexate and infliximab infusions at 5 mg/kg every 4 weeks with subjective improvement in vision, resolution of subretinal fluid on repeat OCT exam in 1 year, and stabilization of skin and ear disease. (Fig. 1C).

Fig. 1
figure 1

A OCT showing abnormal foveal contour and intraretinal fluid. B Side view of the face showing hypopigmented skin lesions involving the forehead, nose, and cheek. C OCT with a resolution of subretinal fluid

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VKHD is a rare systemic inflammatory disease that involves melanocyte-rich tissues, such as the eye, inner ear, meninges, skin, and hair which predominantly affects females in the second to fifth decades of life [1]. The disease course usually follows four phases. The first phase is prodromic and may mimic viral aseptic meningitis followed by an acute uveitic phase that is characterized by diffuse choroidal inflammation, edema of the optic disk, and hyperfluorescent leaking dots on FA [2]. Choroid and skin depigmentation develop later during the convalescent phase with classic sunset glow fundus. Overall, the prognosis of VKHD is favorable with the use of glucocorticoids and other immunosuppressive therapy.