Behcet Syndrome

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Learning objectives

  • Understand the aetiology and risk factors
  • Be able to recognise the clinical presentation
  • Discuss diagnostics and treatment strategies


Behcet's disease is a multisystem, chronic-relapsing vasculitis but its target is predominantly the venous system. It is rare but commoner in the populations emanating from the Middle East, Far East and the Mediterranean.


There are a constellation of typical findings - recurrent oral aphthous ulcerations along with genital ulcers and classical eye inflammatory changes such as uveitis and retinal vasculitis. There are typical skin lesions (erythema nodosum) or a positive pathergy test result. However, the diagnosis is entirely clinical as there is no definitive test. Brain involvement is seen in about a third with stroke and headache. There may be brainstem involvement and meningoencephalitis. Cerebral venous sinus thrombosis may occur.


  • Bloods FBC, U&E, LFTs, CRP, ESR
  • Pathergy test involves using a sterile needle to cause a small skin lesion which develops as a sign of skin hyperreactivity within 48 hours.
  • CT/MRI may show brain lesions. CTV or MRV for suspected CVST.


Management has been with various combinations of drugs such as colchicine, thalidomide, dapsone, azathioprine, interferon-alpha and etanercept as well as oral anticoagulation. In neurosarcoid high dose steroids and immunosuppressives may be used. In aggressive disease cyclophosphamide and methotrexate and interferon-alpha, or azathioprine may be considered. Biological agents may also be considered.