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

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