Abstract
A patient with a remote diagnosis of Vogt-Koyanagi-Harada (VKH) syndrome presented for ophthalmic reassessment before initiation of systemic therapy after hospitalization for progressive fatigue and unintentional weight loss. The prior ocular episode, occurring five years earlier, was characterized by substantial unilateral vision loss and tinnitus and had been treated with adalimumab. On presentation, best-corrected visual acuity was 20/30-2 in the right eye and 20/20 in the left eye, with no active intraocular inflammation. Optical coherence tomography demonstrated a peripapillary and macular choroidal lesion in the right eye with choroidal thickening and hyperreflectivity, and indocyanine green angiography showed a corresponding lesion with calcification and no intrinsic tumor vasculature. The lesion was absent on prior imaging from 2023. Systemic evaluation demonstrated widespread adenopathy and infiltrative soft-tissue disease with elevated inflammatory markers. Excisional axillary lymph-node biopsy revealed numerous histiocytes with emperipolesis, positive S100, CD68, and cyclin D1 immunostaining, and negative ALK-1 staining, establishing the diagnosis of Rosai-Dorfman disease. In the context of systemic disease, the choroidal lesion was reinterpreted as ocular Rosai-Dorfman involvement with dystrophic calcification, and the remote ocular episode was considered a likely earlier manifestation rather than VKH. The patient demonstrated marked symptomatic improvement with high-dose systemic prednisone. Given extensive systemic disease, transition to cobimetinib, a MEK inhibitor, was planned. Baseline and serial ophthalmic examinations, optical coherence tomography, fundus photography, ultrasonography, and visual-field testing were planned to monitor ocular disease response and MEK-inhibitor-associated toxicity.
Presentation Date: 09/17/2026
Issue Date: 09/18/2026
