Published in:
Open Access 01-12-2012 | Review
Behçet's disease
Published in: Orphanet Journal of Rare Diseases | Issue 1/2012
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Definition of the disease
Behçet disease (BD) is a chronic, relapsing, multisystemic disorder characterized by mucocutaneous, ocular, vascular and central nervous system manifestations.
Epidemiology
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.
Clinical description
The clinical spectrum includes oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations.
Etiology
The etiopathogenesis of the disease remains unknown, although genetic predisposition, environmental factors and immunological abnormalities have been implicated.
Diagnostic 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.
Management
Treatment is symptomatic using steroids and immunomodulatory therapy. It is efficient depending on the rapidity of initiation, the compliance, and the duration of therapy.
Prognosis
The prognosis is severe due to the ocular, neurological and arterial involvement.