Behcet's syndrome is a systemic venulitis.
The aetiology of Behcet's disease is not fully understood, however, a combination of genetic and environmental factors may play a role (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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