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Open Access Highly Accessed Review

Behçet's disease

David Saadoun* and Bertrand Wechsler

Author Affiliations

Service de Médecine Interne 2, Centre de Référence Maladies Autoimmunes rares, CHU Pitié-Salpêtrière, 83 Bd de l'Hôpital, 75013, Paris, France

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Orphanet Journal of Rare Diseases 2012, 7:20  doi:10.1186/1750-1172-7-20

Published: 12 April 2012

Abstract

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.

Keywords:
Behçet's disease; Uveitis; Vasculitis; Autoimmunity