Definition:

Vogt-Koyanagi-Harada syndrome (VKHS), initially described as an uveomeningoencephalitic syndrome, is a rare systemic autoimmune disease that targets melanocyte-rich tissues, such as the eye, inner ear, meninges, skin and hair. This disease is characterized by panuveitis, often associated with neurologic and cutaneous manifestations, including headache, hearing loss, vitiligo, and poliosis. VKHS is more common in individuals of pigmented skin, such as Asians, Middle Easterners, Hispanics and Native Americans. Although the exact etiology of VKHS remains unclear, it has also been postulated that such an autoimmune response might be triggered by an infectious agent in a genetically susceptible individual. Several studies have demonstrated that HLA-DR4 is strongly associated with VKHD.