(Translated by https://www.hiragana.jp/)
KEGG DISEASE: Vogt-Koyanagi-Harada syndrome
KEGG   DISEASE: Vogt-Koyanagi-Harada syndrome
Entry
H01504                      Disease                                
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
Brite
Human diseases in ICD-11 classification [BR:br08403]
 14 Diseases of the skin
  Skin disorders involving specific cutaneous structures
   Disorders of the epidermis and epidermal appendages
    Disorders of skin colour
     ED63  Acquired hypomelanotic disorders
      H01504  Vogt-Koyanagi-Harada syndrome
Pathway
hsa04640  Hematopoietic cell lineage
hsa04672  Intestinal immune network for IgA production
hsa04658  Th1 and Th2 cell differentiation
hsa04659  Th17 cell differentiation
hsa04612  Antigen processing and presentation
Gene
HLA-DRB1 [HSA:3123] [KO:K06752]
HLA-DRB4 [HSA:3126] [KO:K06752]
Other DBs
ICD-11: ED63.3
ICD-10: H20.8 H30.8
MeSH: D014607
Reference
  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
LinkDB

» Japanese version

DBGET integrated database retrieval system