Behçet disease is a form of vasculitis that can lead to ulceration and other lesions. It can be interpreted as a chronic disturbance in the body’s immune system that becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects blood vessels, usually the small ones.
Occurring most frequently in the Middle East, Behçet's is endemic among populations clustered along the historic silk road. Young men are most often affected. Current prevalence is estimated to range from a low of 0.3 per 100,000 in Northern Europe to a peak of 16 to 100 per 100,000 in Iran.
An estimated 15,000 to 20,000 Americans have been diagnosed with this disease.
In the UK, it is estimated to have about 1 case for every 100,000 people.
There must be:
Along with 2 out of the next 4 hallmark symptoms:
- HLA B51 positive
- Living in Middle East to South Eastern Siberia (Silk road) because of having triple probability being HLA B51 positive
GENE INVOLVED IN PATHOGENESIS: HLA B51
1) Mouth and genital ulcers:
2) Skin and articular manifestations:
3) Vascular, ocular, intestinal and Neurologic symptoms: