KEGG   DISEASE: フォークト・小柳・原田病
エントリ  
H01504                                                             
名称    
フォークト・小柳・原田病;
ぶどう膜髄膜脳炎症候群
概要    
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.
カテゴリ  
免疫系疾患; 神経系疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 14 皮膚の疾患
  特定の皮膚構造が関与する皮膚疾患
   表皮及び表皮付属器の疾患
    色素異常症
     ED63  後天性色素減少症
      H01504  フォークト・小柳・原田病
パスウェイ 
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
病因遺伝子 
HLA-DRB1 [HSA:3123] [KO:K06752]
HLA-DRB4 [HSA:3126] [KO:K06752]
リンク   
ICD-11: ED63.3
MeSH: D014607
文献    
  著者
Lavezzo MM, Sakata VM, Morita C, Rodriguez EE, Abdallah SF, da Silva FT, Hirata CE, Yamamoto JH
  タイトル
Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes.
  雑誌
Orphanet J Rare Dis 11:29 (2016)
DOI:10.1186/s13023-016-0412-4
LinkDB    

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