| H01504 | |
| H number | H01504 |
| Name | Vogt-Koyanagi-Harada syndrome; Vogt-Koyanagi-Harada disease; Uveomeningoencephalitic syndrome |
| Description | 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. |
| Category | Immune system disease; Nervous system disease |
| Network | - |
| Gene | HLA-DRB1 [HSA:3123] [KO:K06752] HLA-DRB4 [HSA:3126] [KO:K06752] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | - |
| Other DBs | ICD-11: ED63.3 ICD-10: H20.8 H30.8 MeSH: D014607 |
| Reference | PMID:27008848 AUTHORS Lavezzo MM, Sakata VM, Morita C, Rodriguez EE, Abdallah SF, da Silva FT, Hirata CE, Yamamoto JH TITLE Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes. JOURNAL Orphanet J Rare Dis 11:29 (2016) DOI:10.1186/s13023-016-0412-4 |