This site is intended for healthcare professionals
Last reviewed dd mmm yyyy. Last edited dd mmm yyyy
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
Anterior uveitis, posterior uveitis, or cells in the vitreous on slit lamp examination; or retinal vasculitis detected by an ophthalmologist
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:
Positive pathergy test (a)
(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)