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Behcet’s Syndrome

Definition - Beh|et’s syndrome is a multisystem disorder with a world wide distribution that mainly affects young adult presenting with recurrent oral and genital ulcerations as well as ocular involvement.
HLA B 5 & HLA 51. (Ref. Hari. 18th ed., Pg-2802)
Clinical features - Diagnostic Criteria of Beh|et’s Disease = One Major & Two minor criteria’s.
Major criteria - Recurrent oral ulceration
Minor Criteria -
  1. Recurrent genital ulceration
  2. Eye lesions (Ant and post uveitis) can progress rapidly to blindness.
  3. Skin lesions (Erythema nodosum, Pseudofolliculitis)
  4. Pathergy test (Nonspecific skin inflammatory reactivity to any scratches or intradermal saline injection
Other features are:
  1. Deep vein thrombosis
  2. Superficial thrombophlebitis
  3. Arthritis – Nondeforming, mainly involve knees and ankle.
  4. Iv. CNS involvement – mainly in the parenchymal has a serious prognosis (CNS – Beh|et’s syndrome)
Important Points
  1. Myocarditis is not a feature.
  2. In Beh|et syndrome there is Panuveitis i.e. there is involvement of both anterior & posterior uvea.
Pathogenesis (Ref. Hari. 18th ed., Pg-2801)
Circulating autoantibodies against -enolase of endothelial cells and anti–Saccharomyces cerevisiae antibodies (ASCA—characteristic of Crohn's disease) are found to be present in the later stages of the disease.
  1. Mucous membrane involvement may respond to topical glucocorticoids in the form of mouthwash of paste.
  2. In more serious cases thalidomide (100 mg/d) is effective. (AIIMS Nov 2012)
  3. Aspirin for thrombophlebitis
  4. Steroid and azathioprine for uveitis and CNS Beh|et syndrome .
  5. Interferon is very effective for uveitis and CNS Beh|et syndrome. (Ref. Hari. 18th ed., Pg-2802)
Extra Edge:
Other Uses of thalidomide
  1. Amyloidosis,
  2. Behcet syndrome,
  3. Chronic idiopathic myelofibrosis, 
  4. Inflammatory bowel disease,
  5. Lepra reactions,
  6. Multiple myeloma, 
  7. Myelodysplasia,
  8. Sarcoidosis.

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