Service de Médecine Interne 2, Centre de Référence Maladies Autoimmunes rares, CHU Pitié-Salpêtrière, 83 Bd de l'Hôpital, 75013, Paris, France
Orphanet Journal of Rare Diseases 2012, 7:20 doi:10.1186/1750-1172-7-20Published: 12 April 2012
Definition of the disease
Behçet disease (BD) is a chronic, relapsing, multisystemic disorder characterized by mucocutaneous, ocular, vascular and central nervous system manifestations.
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.
The clinical spectrum includes oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations.
The etiopathogenesis of the disease remains unknown, although genetic predisposition, environmental factors and immunological abnormalities have been implicated.
Diagnosis is only based on clinical criteria.
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.
Treatment is symptomatic using steroids and immunomodulatory therapy. It is efficient depending on the rapidity of initiation, the compliance, and the duration of therapy.
The prognosis is severe due to the ocular, neurological and arterial involvement.