Behcet's disease (BD) is a chronic multisystem venulitis of unknown aetiology.
It is characterized by:
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's 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).
References:
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