ICBD: International consensus criteria for the diagnosis of Behçet's disease.
Select Criteria
Select the clinical manifestations present to evaluate ICBD diagnostic criteria.
Guidelines & Evidence
Verified
Last Review: 2026
When to Use
When to Use
Classification of patients with suspected Behçet disease
Standardising diagnosis for clinical research and registry enrolment
Evaluating patients with recurrent oral ulcers plus systemic features
Differentiating Behçet disease from other vasculitides and autoinflammatory syndromes
Patient Population
Adults and children presenting with recurrent oral ulcers and/or other clinical features suggestive of Behçet disease. The International Criteria for Behçet Disease (ICBD) was developed from an international data-driven collaboration and has superseded the older International Study Group (ISG) criteria.
Prerequisites
Recurrent oral ulceration is the most common presenting feature and is present in > 95% of patients. However, the ICBD does not require oral ulcers as a mandatory criterion, unlike the older ISG criteria. The scoring system allows classification solely from other organ involvement if oral ulcers are absent.
How it Works
Point System
| Manifestation | Points |
|---|---|
| Ocular lesions (uveitis, retinal vasculitis) | 2 |
| Genital ulcers (recurrent) | 2 |
| Oral ulcers (recurrent, ≥ 3 episodes in 12 months) | 2 |
| Skin lesions (erythema nodosum, pseudofolliculitis, acneiform lesions) | 1 |
| Vascular manifestations (venous thrombosis, arterial thrombosis/aneurysm) | 1 |
| Neurological manifestations (parenchymal or non-parenchymal) | 1 |
| Pathergy test positive (optional, may not be performed) | 1 |
Interpretation
Total score ≥ 4 points = classified as having Behçet disease. The pathergy test is optional; when not performed, the maximum possible score is 10. Sensitivity is approximately 95% and specificity approximately 90% compared to expert clinical diagnosis.
Pathergy Test
The pathergy test involves pricking the forearm skin with a sterile 20-gauge needle and assessing the site 24–48 hours later for a papular or pustular reaction (typically > 2 mm). The test is more frequently positive in patients of Turkish, Middle Eastern, and Asian descent and is less commonly positive in Northern European or American patients.
Clinical Pearls
Clinical Application
The ICBD criteria were designed to be more sensitive than the older International Study Group criteria, especially in capturing patients with predominantly parenchymal neurological disease, vascular disease, or those without oral ulcers. In clinical practice, the ICBD should be applied by an experienced clinician who can differentiate Behçet-related eye inflammation from other causes of uveitis and Behçet-related skin lesions from common acne.
Differential Diagnosis
Recurrent aphthous stomatitis — oral ulcers without systemic features
Crohn disease — intestinal ulcers, perianal disease, granulomas on biopsy
Systemic lupus erythematosus — oral ulcers with serology and other ACR criteria
Vogt-Koyanagi-Harada disease — uveitis with neurological features but negative pathergy
PFAPA syndrome — periodic fever, aphthous ulcers, pharyngitis in children
ANCA-associated vasculitis — necrotising inflammation with PR3/MPO antibodies
Pitfalls to Avoid
Oral ulcers are common in the general population; document ≥ 3 episodes in 12 months
Genital ulcers must be recurrent and documented on examination, not just reported
Ocular lesions require ophthalmological confirmation; conjunctivitis is not scored
Skin lesions from acne vulgaris in adolescents should be distinguished from Behçet-related lesions
The pathergy test has variable sensitivity across ethnic groups; a negative test does not exclude Behçet
Next Steps
Score ≥ 4 (Classified as Behçet)
01
Refer to rheumatology or appropriate specialist for comprehensive management
02
Obtain baseline ophthalmology assessment (even if eyes are asymptomatic)
03
Screen for vascular involvement: Doppler ultrasound of extremities if clinically indicated
04
Consider HLA-B51 testing (associated but not diagnostic; more common in certain populations)
05
Initiate treatment based on organ involvement: colchicine for mucocutaneous, steroids/immunosuppressants for organ-threatening disease
Score < 4 (Not Classified)
01
Re-evaluate for alternative diagnoses
02
Monitor prospectively, as Behçet manifestations may evolve over months to years
03
Consider periodic reassessment if new symptoms arise
04
Document the specific features present for future re-evaluation
The Evidence
Key Evidence
ICBD was derived from a multi-national dataset of 2,556 patients from 27 countries
Demonstrated superior sensitivity (94.8%) compared to ISG criteria (85.7%) with comparable specificity
Retrospective and prospective validation cohorts confirmed the ≥ 4 threshold as optimal
The criteria are endorsed by the International Team for the Revision of the International Criteria for BD (ITR-ICBD)
Primary Reference
The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria.
International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD) • Journal of the European Academy of Dermatology and Venereology. 2014;28(3):338-47
The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria.
Davatchi F et al. • Journal of the European Academy of Dermatology and Venereology. 2014;28(3):338-47
Origins & History
Development
The ICBD was developed by the International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD), a collaboration involving 27 countries. The criteria were published in 2014 and were designed to improve upon the 1990 International Study Group (ISG) criteria by increasing sensitivity, particularly for patients with vascular, neurologic, and gastrointestinal involvement. The point-based system replaced the older categorical approach and better reflects the variable expressivity of Behçet disease across different ethnic populations.
Last Comprehensive Review: 2026