KEGG   DISEASE: Eosinophilic fasciitis
Entry
H01693                      Disease                                
Name
Eosinophilic fasciitis;
Diffuse fasciitis
Description
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.
Category
Musculoskeletal disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 04 Diseases of the immune system
  Nonorgan specific systemic autoimmune disorders
   4A43  Overlap or undifferentiated nonorgan specific systemic autoimmune disease
    H01693  Eosinophilic fasciitis
Comment
See also H01493 Localized Scleroderma.
Other DBs
ICD-11: 4A43.4
MeSH: C562487
OMIM: 226350
Reference
  Authors
Pinal-Fernandez I, Selva-O' Callaghan A, Grau JM
  Title
Diagnosis and classification of eosinophilic fasciitis.
  Journal
Autoimmun Rev 13:379-82 (2014)
DOI:10.1016/j.autrev.2014.01.019
Reference
  Authors
Oliveira M, Patinha F, Marinho A
  Title
Eosinophilic fasciitis after parasite infection.
  Journal
Reumatologia 54:38-41 (2016)
DOI:10.5114/reum.2016.58761
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