Behçet’s syndrome: providing integrated care

Figures & data

Table 1 Diagnostic criteria sets for BS, ISG and ICBD

ISG criteriaa	Definition	Revised ICBD criteriab	Score
Recurrent OUs	Major/minor aphthous or herpetiform ulceration (at least 3 times a year)	Oral aphthous lesions	2
Recurrent GUs	Aphthous ulcer or scarring	Genital aphthous lesions	2
Ocular involvement	Anterior/posterior uveitis or cells in vitreus or retinal vasculitis	Ocular involvement	2
Skin lesions	EN, pseudofolliculitis, PPL or acneiform nodules in post-adolescent patient not on steroid therapy	Skin lesions	1
Positive pathergy test	Read by physician at 24–48 hours	Vascular involvement	1
		CNS involvement	1
		Positive pathergy test	1

Notes:

a BS diagnosis is made in the presence of recurrent OUs and 2 additional criteria. OUs, GUs and EN may be reported by the patient or physician, whereas skin lesions other than EN must be observed by physician.

b Point score system >3 indicates BS diagnosis. Pathergy test is not involved in the primary scoring system. However, given its specificity for BS, when pathergy test is done, extra point may be assigned.

Abbreviations: BS, Behçet’s syndrome; ISG, The International Study Group; ICBD, The International Criteria for Behçet’s Disease; OU, oral ulcer; GU, genital ulcer; EN, erythema nodosum; PPL, papulopustular lesions; CNS, central nervous system.

Figure 1 Mucocutaneous lesions of Behçet’s syndrome.

Notes: (A) OU; (B) GU with a scar; (C) PPL; (D) acne with arthritis; (E) EN and (F) leg ulcers.
Abbreviations: OU, oral ulcer; GU, genital ulcer; PPL, papulopustular lesions; EN, erythema nodosum.

Figure 2 (A, B) Axial FLAIR-T2 images reveal hyperintense lesion in the brainstem (arrow), (C) axial T1 gadolinium sequence shows gadolinium enhancement (arrow) and (D) prominent brainstem atrophy (arrow) on the follow-up MRI after 3 years.

Figure 3 Endoscopic appearance of round ulcers of GI involvement of BS.

Notes: (A, B) Some of these ulcers can be deep with elevated edges and (C, D) some are shallow.
Abbreviations: GI, gastrointestinal involvement; BS, Behçet’s syndrome.