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Behcet's syndrome

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Behcet's disease (BD) is a chronic multisystem venulitis of unknown aetiology.

It is characterized by:

  • arthritis
  • iritis
  • recurrent oral and genital ulcers
  • neurological disease

It has high prevalence in Turkey, Japan, and Iran, and lower prevalence in North American and northern European populations. Incidence is higher in males in the high prevalence areas, but sex distribution varies in other countries (1).

International Study Group for Behcet's disease (ISG) criteria for the diagnosis of Behcet disease (1,2).

Recurrent oral ulceration

Minor aphthous, major aphthous, or herpetiform ulcers observed by the physician or patient, which have recurred at least 3 times over a 12-month period

Plus any 2 of the following:

Recurrent genital ulceration

Aphthous ulceration or scarring observed by the physician or patient

Eye lesions

Anterior uveitis, posterior uveitis, or cells in the vitreous on slit lamp examination; or retinal vasculitis detected by an ophthalmologist

Skin lesions

Erythema nodosum observed by the physician or patient, pseudofolliculitis, or papulopustular lesions; or acneiform nodules observed by the physician in a postadolescent patient who is not receiving corticosteroids

Positive pathergy test

Test interpreted as positive by the physician at 24-48 h

The International Criteria for Behcet's disease (ICBD) was developed because of low sensitivity of ISG clinical diagnostic criteria (3).

The ICBD criteria are outlined below:

Sign/Symptom

Points

Ocular lesions

2

Genital aphthosis

2

Oral aphthosis

2

Skin lesions

1

Neurological manifestations

1

Vascular manifestations

1

Positive pathergy test (a)

1

(a) Pathergy test is optional. Where a pathergy test is conducted, 1 extra point may be added for a positive result

A patient scoring >= 4 points is classified as having Behcet's disease (3)

A study derived from multinational data showed that the ICBD criteria exhibits much improved sensitivity over the ISG criteria while maintaining reasonable specificity (3)

Reference:


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The content herein is provided for informational purposes and does not replace the need to apply professional clinical judgement when diagnosing or treating any medical condition. A licensed medical practitioner should be consulted for diagnosis and treatment of any and all medical conditions.

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