Hulusi Behçet, an Istanbul native, originally identified the condition in three individuals in 1937. These patients had uveitis, erythema nodosum, and oral and vaginal ulcerations. Later, additional clinical traits were discovered and were included in the disease spectrum. [1] Behcet disease is an unidentified auto-inflammatory systemic vasculitis. Malignant aphthosis and Behcet syndrome are other names for it.
Behcet's Syndrome does not have a mendelian inheritance pattern, but its increased prevalence along the "Silk Route" and familial aggregation point to a genetic component. The most common relationship is with HLA-B51/B5 carriers, who are more likely than non-carriers to develop Behcet disease. HLA B51 is a genetic variant that is frequently seen in populations from Turkey, the Middle East, and Japan.
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