Behçet Disease
Diseases

Edoardo Mantovani
Matteo Dalmasso

DEFINITION

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.

EPIDEMIOLOGY:

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.

SYMPTOMS

DIAGNOSIS:

There must be:

• Oral ulcers

Along with 2 out of the next 4 hallmark symptoms:

• Genital ulcers
• Skin lesions
• Eye inflammation
• Pathergy reaction
  

RISK FACTOR:

• Genetic
  • HLA B51 positive
  • Living in Middle East to South Eastern Siberia (Silk road) because of having triple probability being HLA B51 positive

• Acquired
  • Infection of certain strains of Streptococcus sanguinis

PATHOGENESIS

GENE INVOLVED IN PATHOGENESIS: HLA B51

COMPLICATIONS

THERAPY:

1) Mouth and genital ulcers:

• Mild symptoms:
  • Topic applications of corticosteroids

• Severe symptoms:
  • Colchicine 1-1,5 mg/die
  • Azatiophrine
  • Thalidomide 150-300 mg/die

2) Skin and articular manifestations:

• Colchicine + prednisone 12,5-25 mg/die

3) Vascular, ocular, intestinal and Neurologic symptoms:

• Azatiophrine
• Ciclosporin
• Cyclophosphamide
• Chlorambucil