Behcet's

• Behcet's disease is a complex inflammatory condition affecting the blood vessels and tissues, can affect other areas:

  • Eyes
  • Skin
  • GI system
  • Lungs
  • MSK
  • CNS
• The main features are recurrent oral and genital ulcers
• There is a link with the HLA B51 gene
• Mouth ulcers are very common and have a long list of possible causes:

  • Simple aphthous ulcers are very common without any associated disease
  • Squamous cell carcinoma
  • Herpes simplex virus
  • Hand, foot and mouth disease (coxsackie A virus)
  • Inflammatory bowel disease (e.g., Crohn’s disease)
  • Coeliac disease
  • Connective tissue diseases (e.g., rheumatoid arthritis and systemic lupus erythematosus)
  • Vitamin deficiency (e.g., iron, B12, folate and vitamin D)
  • HIV
• Behcet's ulcers:

  • Oral ulcers expected at least 3 times per year
  • Painful, sharply circumscribed erosions with a red halo occurring on the oral mucosa
  • Heal over 2-4 weeks
  • Genital ulcers are similar in appearance to oral ulcers
  • May leave a scar
• Other organs affected:

  • Skin e.g. erythema nodosum and papules/pustules similar to acne
  • Eyes - anterior or posterior uveitis, retinal vasculitis and retinal artery/vein thrombosis
  • MSK - stiffness, arthralgia and oligo-arthritis
  • GI - ulceration in the ileum, caecum and ascending colon
  • CNS - aseptic meningitis and cerebral venous sinus thrombosis
  • Arteries - aneurysms
  • Veins - DVT and Budd-Chiari syndrome
• The pathergy test involves using a sterile needle to make multiple pricks on the forearm. The area is reviewed 24-48 hours later to look for erythema (redness) and induration (thickening), indicating non-specific skin hypersensitivity. A positive result can indicate Behçet’s disease, Sweet’s syndrome or pyoderma gangrenosum.
• Behcet's disease is a clinical diagnosis
• Management:

  • Topical steroids for mouth ulcers e.g. soluble betamethasone tablets
  • Systemic steroids e.g. oral prednisolone
  • Colchicine can be helpful with symptoms - reduce joint inflammation
  • Topical anaesthetics for genital ulcers e.g. lidocaine ointment
  • Immunosuppressants e.g. azathioprine
  • Biologic therapy e.g. infliximab
• Behçet’s disease takes a relapsing-remitting course. Patients may go into complete remission. Most patients have a normal life expectancy.
• Major complications:

  • Vision loss
  • Ruptured pulmonary aneurysms
  • Bowel perforation
  • Neuro-Behcet's disease