Vogt-Koyanagi-Harada disease in a patient with retinitis pigmentosa in one eye and pigmented paravenous retinochoroidal atrophy in the other eye.

PURPOSE: To report a case of Vogt-Koyanagi-Harada (VKH) disease in a patient with retinitis pigmentosa (RP) in the right eye and pigmented paravenous retinochoroidal atrophy (PPRCA) in the left eye.

OBSERVATIONS: A 32-year-old woman with a history of RP visited our hospital with blurred vision in the left eye. She had a headache for four days before the onset of vision loss. Slit-lamp examination revealed bilateral inflammation of the anterior chamber and vitreous. Fundus examination revealed atrophy of the retinal pigment epithelium (RPE) and diffuse bone spicule pigmentation in the right eye, as well as retinochoroidal atrophy in the peripapillary region and extending along the retinal veins, accompanied by bone spicule pigmentation in the left eye. Optical coherence tomography (OCT) demonstrated serous retinal detachment (SRD) with hyperreflective material only in the left eye and increased choroidal thickness (CT) around the fovea in both eyes. Cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis. The patient was diagnosed with incomplete VKH disease. After treatment with methylprednisolone, SRD in the left eye completely disappeared, and CT decreased bilaterally.

CONCLUSIONS AND

IMPORTANCE

SRD, one of the major characteristic symptoms of VKH disease, may not be common in patients with both RP and VKH disease. VKH disease is associated with meningitis, vitiligo, and hearing loss, but signs other than meningitis are less frequent. However, in RP patients with uveitis, examination of the patient for these non-ocular signs may be needed to diagnose VKH disease.