Incomplete Vogt-Koyanagi-Harada Syndrome Presenting With Sunset Glow Fundus, Vitiligo, Preserved Vision, and Incidental CA 19-9 Elevation.
Nicolas Nicolaou, Antigoni Koukkoulli, Loukiana Tsierkezou
Abstract
Open AccessVogt-Koyanagi-Harada (VKH) disease is a T cell-mediated autoimmune attack on melanocytes in the eyes, ears, skin, and central nervous system. It is characterized by granulomatous panuveitis and often results in significant visual loss. We report a 60-year-old woman referred for suspected chorioretinal inflammation following one month of treatment for anterior uveitis. Remarkably, visual acuity was preserved at 6/6 in both eyes. There was no history of ocular surgery or trauma. Examination revealed trace anterior chamber cells and mild anisocoria. Choroidal folds were present without serous retinal detachment, and the inflammation resolved with topical corticosteroids. At five months, progressive choroidal hypopigmentation resulted in a sunset glow fundus. As no neurological or auditory features were present, the diagnosis initially suggested probable VKH. However, the later development of a single forehead vitiligo patch led to the diagnosis of incomplete VKH. During the acute uveitic stage, a benign liver mass was identified alongside elevated liver enzymes and CA 19-9 tumor marker, which subsequently normalized. Ophthalmologists should be aware of atypical presentations of VKH, particularly in older adults, where systemic symptoms may be mild, absent, or delayed. Vision can remain preserved in the absence of serous retinal detachment. Although the precise triggers of VKH remain unclear, immunological activation has been associated with factors such as immune checkpoint inhibitors, vaccinations, viral infections, and coexisting autoimmune diseases. In this case, a coincidental liver mass was observed, with a transient elevation in tumor marker and liver enzymes. Further case reports are needed to clarify whether such immunoinflammatory changes play a role in initiating autoimmune VKH or represent incidental findings.