Description

• Behçet syndrome is a systemic inflammatory disease of unknown etiology. It presents as a vasculitis of small and large vessels of both arterial and venous systems. It is a chronic disease characterized by oral and genital mucocutaneous ulcerations, skin rashes, arthritis, thrombophlebitis, uveitis, colitis, and neurologic symptoms.
• Synonym(s): Mucocutaneous ocular syndrome; Franceschetti-Valero syndrome, Adamantiades' syndrome

• Thalidomide for treatment contraindicated in pregnancy
• Possible increase in thrombosis and fetal demise

Epidemiology

• Predominant age: 3rd–4th decades; rare in pediatric and geriatric populations
• Predominant gender: Male = Female, with males affected more severely, especially in the Middle East

• Rare in the US and northern Europe; endemic in Japan, the Middle East, and the Mediterranean region. The disease is more prevalent in the region of the Silk Road linking China to Italy (1).
• 1/100,000 population in the US
• In other countries, per 100,000:
  • Turkey: 20–420
  • Islamic Republic of Iran: 16–100
  • Kingdom of Saudi Arabia: 20
  • Japan: 10
  • Northern Europe: 3

Risk Factors

HLA-B51/B5 carriage predominates in males; it is associated with moderately higher prevalences of genital ulcers, ocular and skin manifestations, and a decreased prevalence of GI involvement.

Etiology

Unknown: Classified as systemic vasculitis; associated with HLA-B51; possible immune response to ubiquitous heat-shock protein. Possible infectious causes: Herpes simplex virus (HSV), streptococcus; reportedly associated with HIV infection; possible environmental toxin: Heavy metals, pesticides, possibly English walnuts or ginkgo nuts

Commonly Associated Conditions

• Amyloid
• Myelodysplastic syndrome and trisomy 8