Behçet's disease diagnostic study of choice

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hamid Qazi, MD, BSc [2]


Currently, there is not a specific test to confirm the diagnosis of Behçet's disease. It is diagnosed clinically by specific patterns of symptoms and repeated outbreaks obtained by a thorough history of the patient's symptoms (outlined below). Behçet's disease is a diagnosis of exclusion, and other chronic inflammatory diseases should be evaluated for. The various inflammatory symptoms do not necessarily occur together, and they will vary in severity.

Diagnostic Study of Choice

Study of choice

  • The pathergy test should be performed when:[1]
    • The patient presented with symptoms of vision problems, mouth sores, and genital ulcers.
    • A positive pathergy reaction (papule >2 mm dia. 24-48 hrs or more after needle-prick) is detected in the patient.
  • Behçet disease is mainly diagnosed based on clinical presentation.

Diagnostic Criteria

There are three levels of certainty for diagnosis:

  • International Study Group diagnostic guidelines (very strict for research purposes)
  • Practical clinical diagnosis (generally agreed pattern but not as strict)
  • 'Suspected' or 'Possible' diagnosis (incomplete pattern of symptoms)
  • International Criteria for Behçet's disease (ICBD)

International Study Group diagnostic guidelines (ISGD)

The ISGD guidelines recommend the following:[2][3]

  • Must have oral (aphthous) ulcers (any shape, size or number at least 3 times in any 12 months), along with 2 out of the next 4 "hallmark" symptoms:
    • Genital ulcers (including anal ulcers and spots in the genital region and swollen testicles or epididymitis in men)
    • Skin lesions (papulo-pustules, folliculitis, erythema nodosum, acne in post-adolescents not on corticosteroids)
    • Eye inflammation (iritis, uveitis, retinal vasculitis, cells in the vitreous)
    • Pathergy reaction (papule >2 mm diameter 24-48 hrs or more after needle-prick)

Practical clinical diagnosis

The practical clinical diagnosis for Behçet disease recommend the following:[1]

'Suspected' or 'Possible' diagnosis

Usually assigned when someone does not have mouth ulcers, or has mouth ulcers but does not have 1 of the 4 hallmark symptoms but has other symptoms and signs of inflammation, and other causes for these have been ruled out.

International Criteria for Behçet's disease (ICBD)


  1. 1.0 1.1 Ferraz MB, Walter SD, Heymann R, Atra E (1995). "Sensitivity and specificity of different diagnostic criteria for Behçet's disease according to the latent class approach". Br J Rheumatol. 34 (10): 932–5. PMID 7582698.
  2. "Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease". Lancet. 335 (8697): 1078–80. 1990. PMID 1970380.
  3. Smith EL, Shmerling RH (1999). "The American College of Rheumatology criteria for the classification of systemic lupus erythematosus: strengths, weaknesses, and opportunities for improvement". Lupus. 8 (8): 586–95. doi:10.1191/096120399680411317. PMID 10568894.
  4. 4.0 4.1 Davatchi F (2012). "Diagnosis/Classification Criteria for Behcet's Disease". Patholog Res Int. 2012: 607921. doi:10.1155/2012/607921. PMC 3180812. PMID 21961081.

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