Vogt Koyanagi Harada Syndrome: A Pandora's Box!

Abstract

Vogt-Koyanagi-Harada syndrome is a rare systemic granulomatous autoimmune condition that affects melanocyte-rich organs including the eyes, inner ears, meninges, skin, and hair. It causes severe bilateral, chronic granulomatous panuveitis associated with serous retinal detachments and disc oedema. The etiology of Vogt-Koyanagi-Harada syndrome is extremely variable. Here, we report 4 cases diagnosed with Vogt-Koyanagi-Harada syndrome, who presented with completely different backgrounds. The first case was a 34M who presented with a 3 months history of diminution of vision with headache and tinnitus. The second case was a 21F who presented with a 1-day history of sudden diminution of vision associated with a headache on day 2 after undergoing an emergency lower segment cesarean section. The third patient was a 30F who was a case of systemic lupus erythematosus associated with type 4 lupus nephritis. She presented with a 2-day history of diminution of vision associated with headache and tinnitus. She had an active herpes zoster infection involving the trunk at the time of presentation. The fourth case was a 30F who presented to us 7 days post-vaccination with a 5-day history of sudden diminution of vision in both eyes. All the patients had choroiditis with exudative retinal detachment confirmed on OCT suggestive of Vogt-Koyanagi-Harada syndrome. This case series throws light on variable etiology and multisystem involvement of Vogt-Koyanagi-Harada syndrome and management criteria. Vogt-Koyanagi-Harada syndrome is an important differential diagnosis in all patients presenting with the sudden blurring of vision with the headache.