What are the diagnostic criteria for Behçet disease?

Updated: Dec 21, 2020
  • Author: Nicole Davey-Ranasinghe, MD; Chief Editor: Herbert S Diamond, MD  more...
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In 1990, the International Study Group (ISG) for Behçet's Disease clarified criteria for the diagnosis of Behçet disease. [3] The ISG group compared the clinical findings of 914 patients with a history of aphthous ulcers with those of controls. Initial criteria for diagnosis require the occurrence of at least three episodes of oral herpetiform or aphthous ulcerations within a 12-month period observed directly by a physician or reported by the patient. To confirm the diagnosis, at least two of the following must also be demonstrated:

  • Recurrent painful genital ulcers that heal with scarring

  • Ophthalmic lesions, including anterior or posterior uveitis, hypopyon, or retinal vasculitis

  • Skin lesions, including erythema nodosum–like lesions, pseudofolliculitis, or papulopustular or acneiform lesions

  • Positive results from pathergy skin testing, defined as the formation of a sterile erythematous papule 2 mm in diameter or larger that appears 48 hours following a skin prick with a sharp sterile needle (22-24 gauge [a dull needle may be used as a control])

See the Behcet's Syndrome International Study Group Criteria calculator.

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