Vogt-Koyanagi-Harada disease is always bilateral: reports of unilateral cases failed to use choroidal investigations showing subclinical involvement of the fellow eye

BACKGROUND/PURPOSE: Vogt-Koyanagi-Harada (VKH) disease is a primary stromal choroiditis and a bilateral granulomatous panuveitis which, if not treated early and properly, could have a deleterious evolution. The purpose of our case report is to demonstrate that “so called” unilateral VKH disease should be investigated further with an Indocyanine Green Angiography (ICGA), in order to detect subclinical choroidal involvement of the other eye. CASE REPORT: We present a case of 42-year old woman who came to see us for second opinion. She had consulted elsewhere for a left uveitis and had been treated with a periocular corticosteroid injection. At presentation she mentioned persistent headaches. Visual acuity on the Snellen scale was 1.0 OD and 0.5 OS. Slit-lamp examination showed granulomatous (rare mutton-fat KPs) signs in her left eye. Laser flare photometry showed a subclinical flare of 17.8 ph/ms OD and a flare of 66.4 ph/ms OS (normal values 3–6 ph/ms). Fundus examination showed left discoloration due to choroidal infiltration with a normal fundus aspect OD. ICGA showed a diffuse choroiditis also in the apparently normal right eye. Lumbar puncture confirmed the diagnosis of VKH and appropriate treatment was introduced. CONCLUSION: VKH disease results from a generalized autoimmune process against melanocyte associated antigens starting in the choroidal stroma. It can be asymmetrical but is always bilateral, as long as investigations such as ICGA, able to detect subclinical choroiditis, are performed.