Box 2.

Diagnostic criteria sets for BD.

Major diagnostic features  GA: genital aphthosis (recurrent painful, sharply defined, round or ovaloid shallow ulceration, including perianal)  OA: oral aphthosis  OM: ocular manifestations  Minor diagnostic features  NM: neurologic manifestations (vascular or parenchymal involvement)  SM: skin manifestations (necrotic folliculitis, acneiform lesions or erythema nodosum)  VM: vascular manifestations (venous thrombosis, arterial thrombosis and arterial aneurysms)

Diagnostic criteria set for paediatric BD

Any three out of the six criteriaPEDBD

Diagnostic criteria set for adult BD

⩾ 4 points (2 points for major, 1 point for minor and 1 point for a positive pathergy test (pustular response to blunt dermal needle prick) test if carried out in at least 90% of patients)ICBD OR OA + 2 out of remaining criteriaISG

BD, Behçet’s disease; GA, genital aphthosis; ICBD, International Criteria for Behçet’s disease;⁸³ ISG, International Study Group;⁸⁴ NM, neurologic manifestations; OA, oral aphthosis; OM, ocular manifestations; PEDBD, paediatric Behçet’s disease; SM, skin manifestations;⁸⁵ VM, vascular manifestations.