What are the Revised International Criteria for Adamantiades Behçets disease?
• Recurrent oral ulceration (one point): recurrent (≥3 times per year) and multiple minor aphthous, major aphthous, or herpetiform ulcerations.
• Recurrent genital ulceration (two points): aphthous ulceration or scarring.
• Eye lesions (two points): anterior uveitis, posterior uveitis, or cells in vitreous on slit lamp examination; or retinal vasculitis.
• Skin lesions (one point): pseudofolliculitis, papulopustular lesions, erythema nodosum-like lesions, acneiform lesions, pyoderma gangrenosum-like lesions.
• Vascular lesions (one point): phlebitis, large vein thrombosis, aneurysm, arterial thrombosis.
• Positive pathergy test (one point): 2 mm erythema 24 to 48 hours after a #20- to 21-gauge needle prick to a depth of 5 mm.
The diagnosis is confirmed in a patient with three or more points. Validation studies show that these criteria have an accuracy of 89% to 97%. Note that pulmonary aneurysms, major venous thrombosis, genital ulcers that scar, hypopyon, and pathergy are highly characteristic of Behçet’s disease in a patient presenting with recurrent oral ulcers. Histopathologic analysis of involved tissues ranges from a neutrophilic vascular reaction to leukocytoclastic vasculitis.
