Bechet's Disease

Question 1

Definition and presentation of Bechet’s disease

Answer 1

• Complex inflammatory condition
• Mouth ulcers
• Genital ulcers
• Skin
  
  • Erythema nodosum
  • Papules and pustules (similar to acne)
  • Vasculitic type rashes
• Eyes
  
  • Anterior or posterior uveitis
  • REtinal vasculitis
  • Retinal haemorrhage
• MSK
  
  • Morning stiffness
  • Arthralgia
  • Oligoarthritis often affecting the knee or ankle - swelling without destruction
• GI
  
  • Inflammation (tends to affect the ileum caecum and ascending colon)
• CNS
  
  • Budd Chiari syndrome
  • DVT
  • Thrombus in pulmonary veins
  • Cerebral venous sinus thrombosis
• Lungs
  
  • Pulmonary artery aneyrusms
• Like with HLAB51 gene

Question 2

Investigation and management of Bechet’s disease

Answer 2

• Pathergy test - sterile needle to scratch skin, assess 24-48hrs later to look for weal 5mm or more in size - positive in Bechet’s, Sweet’s syndrome and pyroderma gangrenosum
• Management involves:
  
  • Topical steroids to mouth ulcers (soluble betamethasone)
  • Systemic steroids (prednisolone)
  • Colchicine as an anti-inflammatory
  • Topical anaesthetics for genital ulcers (lidocaine ointment)
  • Immunosuppresants (azathioprine)
  • Biologic therapy (infliximab)