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Behçet disease (Behçet’s syndrome, Morbus Behçet, silk road disease) (IPA: /b?’t??t/) (enPR: beCHET) is a chronic condition due to disturbances in the body’s immune system. This system, which normally protects the body against infections through controlled inflammation, becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects blood vessels, usually the small ones. As a result, symptoms occur wherever there is a patch of inflammation, and can be anywhere where there is a blood supply.
Behçet disease is named after Hulusi Behçet (1889-1948), the Turkish dermatologist and scientist who first recognized the syndrome in one of his patients in 1924 and reported his research on the disease in Journal of Skin and Venereal Diseases in 1936.[1][2] The name (Morbus Behçet) was formally adopted at the International Congress of Dermatology in Geneva in September 1947.
Symptoms of this disease may have been described by Hippocrates in the 5th century BC, in his 3rd Epidemion-book.[3] Its first modern formal description was published in 1922.[1]
Some sources use the term “Adamantiades’ syndrome” or “Adamandiades-Behçet syndrome”, for the work done by Benediktos Adamantiades.[4] However, the current World Health Organization/ICD-10 standard is “Behçet’s disease”.
In 1991, Saudi Arabian medical researchers described “neuro-Behçet’s disease”,[5] a neurological involvement in Behçet’s disease, considered one of the most devastating manifestations of the disease as described by an Egyptian researcher.[6]
There is no specific pathological test for Behçet disease at present. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks. Other causes for these symptoms have to be ruled out before making the diagnosis. The symptoms do not have to occur together, but can have happened at any time.
There are three levels of certainty for diagnosis:
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