Table 2.

Main diagnostic/classification criteria for Behçet's disease.

Criteria of the International study group for BD (10)

Recurrent Oral Ulceration (Mandatory): Minor aphthous, major aphthous, or herpetiform ulceration observed by physician or patient, which recurred at least 3 times in one 12-month period

Plus 2 of

Recurrent genital ulceration: Aphthous ulceration or scarring, observed by physician or patient

Eye lesions: Anterior uveitis, posterior uveitis, or cells in vitreous on slit lamp examination; or retinal vasculitis observed by ophthalmologist

Skin lesions: Erythema nodosum observed by physician or patient, pseudo folliculitis, or papulopustular lesions; or acneiform nodules observed by physician in post adolescent patients not on corticosteroid treatment

Positive pathergy test: Read by physician at 24–48 h.

International Criteria for Behçet's Disease (11)

• Ocular lesions 2 points      • Genital aphthosis 2 points      • Oral aphthosis 2 points      • Skin lesions 1 point      • Neurological manifestations 1 point      • Vascular manifestations 1 point      • Positive pathergy test* 1 point*       4 or more points are required for diagnosis.

*Pathergy test is optional. If it is performed and resulted as positive, additional 1 point may be added.

Pediatric Criteria for Behçet's Disease (12)

Recurrent oral aphthosis: At least three attacks/year

Genital ulceration or aphthosis: Typically, with scar

Skin involvement: Necrotic folliculitis, acneiform lesions, erythema nodosum

Ocular involvement: Anterior uveitis, posterior uveitis, retinal vasculitis

Neurological signs: With the exception of isolated headaches

Vascular signs: Venous thrombosis, arterial thrombosis, arterial aneurysm

At least 3 criteria are required for the diagnosis.