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Clinical-Pathological Correlation of Vogt-Koyanagi-Harada Disease

Patrick Oellers, MD1; Glenn J. Jaffe, MD1; Alan D. Proia, MD, PhD2
[+] Author Affiliations
1Department of Ophthalmology, Duke University Medical Center, Durham, North Carolina
2Department of Pathology, Duke University Medical Center, Durham, North Carolina
JAMA Ophthalmol. 2016;134(3):343-345. doi:10.1001/jamaophthalmol.2015.5464.
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This case report describes a patient with panuveitis-choroidal effusion in the right eye.

Vogt-Koyanagi-Harada (VKH) disease is a multisystem autoimmune disorder involving the eyes, ears, meninges, and skin. Ophthalmological hallmarks are bilateral panuveitis and serous retinal detachments.1 Histologically, the disease is characterized by inflammation of the choroid.2 We present a case that provides unique insights owing to the direct correlation of multimodal clinical imaging with histology.

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Figure 1.
Clinical Findings

A, Fundus photograph of the right eye revealing optic disc edema and inferior choroidal effusion initially. B, Fundus photograph of the left eye with subretinal fluid 2.5 weeks later. C, Corresponding optical coherence tomography (OCT) of the right eye disclosed an elevated optic nerve (upper image) and subretinal fluid through the left fovea (lower image, corresponding to Figure 1B). D, Fluorescein angiography of the left eye revealing multiple areas of pinpoint hyperfluorescence, which increased throughout the study, and optic nerve head staining (left image, 22 seconds; right image, 3 minutes and 33 seconds). E, Inferotemporal retina of the right eye with choroidal hypopigmentation (“sunset glow” fundus) and pigment clumping 4 months after initial presentation. F, OCT of the left eye 6 months after presentation revealed an ill-defined retinal pigment epithelium and ellipsoid zone layers and a mixed hyperreflective intraretinal and subretinal lesion adjacent to the optic nerve. Visual acuity was 20/40 OD and 20/50 OS.

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Figure 2.
Histopathological Findings

A, Diffuse granulomatous inflammation of the choroid (bar = 100 µm) extended focally into choriocapillaris (inset; bar = 10 µm). B, Loss of choroidal melanocytes (surrounding the asterisk) corresponding to “sunset glow” in Figure 1E (bar = 200 µm). C, Peripapillary subretinal fibrosis and choroidal neovascularization corresponding to optical coherence tomographic image in Figure 1F (bar = 100 µm). D, Dalen-Fuchs nodule (focal aggregate of epithelioid histiocytes admixed with retinal pigment epithelial cells between the Bruch membrane and the retinal pigment epithelium; bar = 25 µm). All slides stained with hematoxylin-eosin.

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