Atypical Vogt-Koyanagi-Harada disease or new uveomeningitic syndrome?

To report on a patient affected by bilateral intermediate uveitis (IU) as the initial sign of an uveomeningitic syndrome.

Thorough history, physical examination and ancillary laboratory and radiological testing were performed in this observational case study.

A 23-year-old Caucasian man developed bilateral IU, primarily diagnosed as "idiopathic" since a detailed etiologic work-up was not indicative of underlying disease. Seven months later, he presented with poliosis and vitiligo. Lumbar puncture revealed cerebrospinal fluid pleocytosis. Optical coherence tomography showed bilateral subclinical macular edema (ME). The visual acuity was still 20/20 in both eyes. Clinical, laboratory and radiological results did not fit into any known syndrome.

According to all the tests performed, the disease in our patient is a uveomeningitic disease with IU and ME which could be interpreted as an atypical form of Vogt-Koyanagi-Harada disease or a new uveomeningitic syndrome because there is no evidence for any other known disease.