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Behcet’s disease

Introduction

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 if typical findings - recurrent oral 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. The pathergy test involves using a sterile needle to cause a small skin lesion which develops as a sign of skin hyperreactivity within 48 hours. However the diagnosis os 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. Cerebral venous sinus thrombosis may occur. 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 immumnosuppresives may be used. In aggressive disease cyclophosphamide and methotrexate and interferon-alpha, or azathioprine may be considered. Biological agents may also be considered.

References

  • Diagnosis and treatment of cerebral vasculitis, Peter Berlit. Ther Adv Neurol Disord (2010) 3(1) 2942

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