KEGG   DISEASE: 好酸球性筋膜炎
エントリ  
H01693                                                             
名称    
好酸球性筋膜炎;
びまん性筋膜炎
概要    
Eosinophilic fasciitis (EF) is a systemic inflammatory disease characterized by symmetrical swelling and skin induration of the arms and/or legs, evolving into a scleroderma-like appearance, accompanied by peripheral blood eosinophilia. The disease is present almost equally in both sexes. The etiology of EF remains uncertain. Hematological, infectious, and autoimmune diseases, as well as intense physical exertion, chemical compounds, drugs, solid neoplasms, and physical factors have been proposed as possible triggers and associated factors. Some researchers have proposed an aberrant immune response as the main pathogenetic mechanism. Some EF patients improve spontaneously without treatment, but in those who do not, glucocorticoids are the mainstay therapy. Methotrexate at low doses is probably the most favored second-line treatment, especially in patients with morphea-like skin lesions.
カテゴリ  
筋骨格疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 04 免疫系の疾患
  非器官特異的全身性自己免疫疾患
   4A43  重複または未分化の非器官特異的全身性自己免疫疾患
    H01693  好酸球性筋膜炎
コメント  
See also H01493 Localized Scleroderma.
リンク   
ICD-11: 4A43.4
MeSH: C562487
OMIM: 226350
文献    
  著者
Pinal-Fernandez I, Selva-O' Callaghan A, Grau JM
  タイトル
Diagnosis and classification of eosinophilic fasciitis.
  雑誌
Autoimmun Rev 13:379-82 (2014)
DOI:10.1016/j.autrev.2014.01.019
文献    
  著者
Oliveira M, Patinha F, Marinho A
  タイトル
Eosinophilic fasciitis after parasite infection.
  雑誌
Reumatologia 54:38-41 (2016)
DOI:10.5114/reum.2016.58761
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