Behçet’s Syndrome

Bechet’s syndrome is a chronic multisystemic inflammatory disorder characterised by recurrent oral and genital ulcers, uveitis, and skin lesions. It is associated with HLA-B5. The diagnosis is clinical.

It is more prevalent in countries along the Silk Road, from Eastern Asia to the Mediterranean Basin. It is named after Hulusi Behçet (1889 – 1948), who was a Turkish dermatologist.

• Risk factors
  • HLA-B51 or HLA-B5
  • Infections, e.g., HSV, can trigger the syndrome
  • Immune dysregulation
• Signs and symptoms
  • Recurrent oral and genital ulcers
  • Uveitis → blindness
  • Retinal vasculitis
  • Optical neuritis
  • Skin lesions
    • Erythema nodosum
    • Papulopustular lesions
  • Arthritis
    • Non-erosive large joint oligoarthropathy
  • Thrombophelbitis
  • Vasculitis
  • Myocarditis and pericarditis
  • Pyramidal signs
  • Colitis (mimics inflammatory bowel disease)
• Investigations
  • Pathergy test
    • Papules form within 48 hours after a needle prick
• Treatment
  • Colchicine or Topical steroids for oral and genital ulcers
  • Topical anaesthetics for relieving pain in ulcers
  • Systemic steroids for ocular or vascular involvement
  • Infliximab for ocular disease unresponsive to topical steroids
  • DMARDs (azathioprine, methotrexate, or cyclosporine) for systemic disease unresponsive to steroids
  • Cyclophosphamide for severe vasculitis or neurological involvement
• Complications
  • Uveitis
  • Vision loss
  • Venous thromboembolism
  • Vasculitis
  • Neuro-Behcet’s syndrome: headache, fever, stiff neck, and neurological deficits
  • Pseudotumour cerebri
  • Gastrointestinal ulceration and perforation