Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?
Table 1
Diagnostic criteria of the International Study Group for Behçet’s disease (1990).
Criteria
Definition
Recurrent oral ulceration
Minor aphthous, major aphthous, or herpetiform ulceration observed by physician or patient, which recurred at least 3 times in one 12-month period.
Plus any two 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, pseudofolliculitis or papulopustular lesions, or acneiform nodules observed by physician in postadolescent patients not on corticosteroid treatment.
Positive pathergy test
Papule/Pustule ≥2 mm surrounded by an erythematous halo, 24–48 h (read by physician) after a blunt needle prick (18–21 gauge) on the intradermal and subcutaneous part of the volar forearm at an angle of 45° to a depth of 5 mm.