title: Behçet's disease.
abstract: DEFINITION OF THE DISEASE: Behçet disease (BD) is a chronic, relapsing, multisystemic disorder characterized by mucocutaneous, ocular, vascular and central nervous system manifestations.
  
  
  BACKGROUND
  BD seems to cluster along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. European cases are often described, not exclusively in the migrant population.
  
  
  METHODS
  The clinical spectrum includes oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations.
  
  
  BACKGROUND
  The etiopathogenesis of the disease remains unknown, although genetic predisposition, environmental factors and immunological abnormalities have been implicated.
  
  
  METHODS
  Diagnosis is only based on clinical criteria. DIFFERRENTIAL DIAGNOSIS: It depends on the clinical presentation of BD, but sarcoidosis, multiple sclerosis, Crohn's disease, Takayasu's arteritis, polychondritis or antiphospholipid syndrome need to be considered.
  
  
  RESULTS
  Treatment is symptomatic using steroids and immunomodulatory therapy. It is efficient depending on the rapidity of initiation, the compliance, and the duration of therapy.
  
  
  CONCLUSIONS
  The prognosis is severe due to the ocular, neurological and arterial involvement.
authors.affiliation: Service de Médecine Interne 2, Centre de Référence Maladies Autoimmunes rares, CHU Pitié-Salpêtrière, 83 Bd de l'Hôpital, 75013, Paris, France. david.saadoun@psl.aphp.fr
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.forename: Bertrand
authors.initials: B
authors.lastname: Wechsler
mesh_terms: D000293:Adolescent; D000328:Adult; D015551:Autoimmunity; D001528:Behcet Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology; D006801:Humans; D056747:Immunomodulation; D035583:Rare Diseases; D013256:Steroids / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D013256:Steroids