Vogt-Koyanagi-Harada disease diagnosed in members of the same family

Abstract

Vogt-Koyanagi-Harada syndrome (VKH syndrome) is a rare granulomatous inflammatory disease that affects melanocyte pigment-producing melanocytes and primarily affects pigmented structures such as the eyes, inner ear, skin, meninges, and hair. VKT is an autoimmune disease, which is primarily a CD4 + Th1 T lymphocyte-mediated aggression to melanocytes. Melanin usually gives color to the skin, hair and eyes. Melanin is also found in the retina, where it plays a role in normal vision. The absence of ocular trauma or previous intraocular surgery differentiates VKHD from sympathetic ophthalmia, its main differential diagnosis. The disease has an acute onset of bilateral blurred vision with hyperemia preceded by flu-like symptoms. The acute uveitic stage is characterized by diffuse choroiditis with serous retinal detachment and optic disc hyperemia and edema. Fluorescein angiography at this stage demonstrates multiple initial hyperfluorescent dots. After the acute uveitic stage, pigmentary changes in the ocular and integumentary system may appear. Ocular findings may be accompanied by lymphocytic meningitis, hearing loss and/or tinnitus in a variable proportion of patients. Prompt diagnosis followed by early, aggressive, and long-term treatment with high-dose corticosteroids is most often followed by good visual results. However, some patients may have chronic uveal inflammation with functional deterioration of the eye.