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Диагностические критерии синдрома Бехчета

Описан в 1937 году турецким дерматологом Hulusi Behçet

International Study Group for Behçet’s Disease Criteria (1989)

Diagnosis requires:  Recurrent oral ulceration: minor aphthous, major aphthous, or herpetiform ulceration observed by physician or by a reliable patient, with recurrence of 3 episodes in one I 2-month period

PLUS > 2/4 of the following: 

I. Recurrent genital ulceration: 

  • aphthous ulceration or scarring, observed by physician or by a reliable patient 

2. Eye lesions: 

  • anterior uveitis, or 
  • posterior uveits or 
  • cells in vitreous on slit lamp examination or 
  • retinal vasculitis observed by an ophthalmologist 

3. Skin lesions: 

  • erythema nodosum, observed by physician or by a reliable patient or 
  • pseudofolliculitis, or 
  • papulopustular lesions; or 
  • acneiform nodules, observed by physician in post-adolescent patients not receiving corticosteroids 

4. Positive pathergy test read by a physician at 24-48 hours: 

  • an indurated erythematous papulc or pustule that is> 2mm at the prick site after the application of a sterile needle or intradermal injection of saline 

N.B.  Findings applicable only in absence of other clinical explanations.

O’Duffy Behçet’s Disease Criteria (I974)

Diagnosis requires:  Recurrent oral aphthous ulcers PLUS 2 of: 

  • Aphthous genital ulceration 
  • Uveitis 
  • Cutaneous “pustular” vasculitis 
  • Synovitis 
  • Meningoencephalitis 

Incomplete form: Recurrent oral aphthous ulcers PLUS > 1 of above criteria  Exclusions:herpes simplex infection, inflammatory bowel disease, Reiter’s syndrome and systemic lupus erythematosus (SLE)

Behçet’s Disease Research Committee of Japan Criteria (I972)

Diagnosis requires: All 4-Major Criteria

Major Criteria:

1. Recurrent aphthous ulceration in the mouth  2. Skin lesions 

  • Erythema nodosum-ilke eruptions 
  • Subcutaneous thromhophlebitis 
  • Hyperirritability of the skin 

3. Eye lesions 

  • Recurrent hypopyon iritis or iridocyclitis 
  • Chorioretinitis 

4. Genital ulcerations  Incomplete type: 3/4 major criteria, or Recurrent hypopyon-iritis or typical chorioretinitis +1 other major criterion

Minor Criteria:

(Any number may present) 

  • Arthritis symptoms (arthralgia, swelling, erythema) 
  • Gastrointestinal lesions (e.g appen(licitis-like pains, melena) 
  • Epididymitis 
  • Vascular lesions (occlusion of blood vessels, aneurysms) 
  • Central nervous system involvement 
  • Brain stem syndrome 
  • Meningo-encephalomyelitic syndrome 
  • Confusional type
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