Behçet's disease

<p>Abstract</p> <p>Definition of the disease</p> <p>Behçet disease (BD) is a chronic, relapsing, multisystemic disorder characterized by mucocutaneous, ocular, vascular and central nervous system manifestations.</p> <p>Epidemiology</p> <p>BD seem...

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Bibliographic Details
Main Authors: Saadoun David, Wechsler Bertrand
Format: Article
Language:English
Published: BMC 2012-04-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Online Access:http://www.ojrd.com/content/7/1/20
Description
Summary:<p>Abstract</p> <p>Definition of the disease</p> <p>Behçet disease (BD) is a chronic, relapsing, multisystemic disorder characterized by mucocutaneous, ocular, vascular and central nervous system manifestations.</p> <p>Epidemiology</p> <p>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.</p> <p>Clinical description</p> <p>The clinical spectrum includes oral and genital ulcerations, uveitis, vascular, neurological, articular, renal and gastrointestinal manifestations.</p> <p>Etiology</p> <p>The etiopathogenesis of the disease remains unknown, although genetic predisposition, environmental factors and immunological abnormalities have been implicated.</p> <p>Diagnostic methods</p> <p>Diagnosis is only based on clinical criteria.</p> <p>Differrential diagnosis</p> <p>It depends <b>on</b> the clinical presentation of BD, but sarcoidosis, multiple sclerosis, Crohn’s disease, Takayasu’s arteritis, polychondritis or antiphospholipid syndrome <b>need to be considered</b>.</p> <p>Management</p> <p>Treatment is symptomatic using steroids and immunomodulatory therapy. It is efficient depending on the rapidity of initiation, the compliance, and the duration of therapy.</p> <p>Prognosis</p> <p>The prognosis is severe due to the ocular, neurological and arterial involvement.</p>
ISSN:1750-1172