Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?

Table 2

Diagnostic criteria of the Behçet’s Disease Research Committee of Japan (2003 revision).

Criteria	Diagnosis
Major manifestations: (1) Recurrent oral aphthous ulceration; (2) Skin lesions; (i) Erythema nodosum; (ii) Subcutaneous thrombophlebitis; (iii) Folliculitis, acne-like lesions; (iv) Cutaneous hypersensitivity; (3) Eye lesions; (i) Iridocyclitis; (ii) Chorioretinitis, retinouveitis; (iii) Definite history of chorioretinitis or retinouveitis; (4) Genital ulcers; Minor manifestations; (1) Arthritis without deformity and ankylosis; (2) Gastrointestinal lesions characterized by ileocecal ulcers; (3) Epididymitis; (4) Vascular lesions; (5) Central nervous system symptoms	(I) Complete type; Four major manifestations appear in the course of the illness.
	(II) Incomplete type; (A) Three major manifestations or 2 major and 2 minor manifestations appear in the course of the illness.; (B) Typical ocular manifestations and one major or two minor manifestations appear in the course of the illness.
	(III) Possible type; Certain major manifestations appear but do not qualify as the incomplete type and typical minor manifestations repeat or worsen.
	(IV) Specific types; (a) Intestinal-BD; (b) Vasculo-BD; (c) Neuro-BD
	Additional assessment for reference (not mandatory); (i) Positive/negative pathergy test.; (ii) Positive/negative prick test with a streptococcus vaccine.; (iii) Inflammatory response (ESR, CRP, WBC, Complement titer).; (iv) Positive/negative HLA-B*51. The type of HLA type should be listed.; (v) Pathologic findings.