BEHÇET’S DISEASE

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  • There is a link to the HLA-B51 allele
  • Clinical features
    • The cardinal clinical feature is recurrent oral ulceration.
    • The international criteria for diagnosis require oral ulceration and any two of the following:
      • genital ulcers
      • defined eye lesions
      • defined skin lesions
      • a positive skin pathergy test
    • Oral ulcers can be aphthous or herpetiform.
    • The eye lesions include an anterior or posterior uveitis or retinal vascular lesions.
    • Cutaneous lesions consist of erythema nodosum, pseudofolliculitis and papulopustular lesions.
    • Other manifestations include
      • a self-limiting peripheral mono- or oligoarthritis affecting knees, ankles, wrists and elbows
      • gastrointestinal symptoms of diarrhoea, abdominal pain and anorexia; pulmonary and renal lesions
      • a brainstem syndrome
      • organic confusional states
      • meningoencephalitis.
    • All the common manifestations are self-limiting except for the ocular attacks.
    • Repeated attacks of uveitis can cause blindness.
    • The pathergy reaction is highly specific to Behçet’s dise
      • Skin injury, by a needle prick for example, leads to papule or pustule formation within 24–48 hours.

Treatment

  • Corticosteroids, immunosuppressive agents and ciclosporin are used for chronic uveitis
  • Colchicine helps erythema nodosum and joint pain.
  • Thalidomide may be useful in erythema nodosum
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