Behçet’s Disease

Hulusi Behçet, a Turkish dermatologist, in 1937 described a chronic relapsing syndrome of oral ulceration, genital ulceration, and uveitis that now bears his name.

Behçet’s disease should be considered in any patient with a systemic inflammatory disease characterized by recurrent orogenital ulcers and uveitis.
Behçet’s disease is the only vasculitis to cause pulmonary aneurysms.
Pathergy at the site of needle sticks is characteristic of Behçet’s disease.

Revised International Criteria for Adamantiades–Behçet’s disease:

Recurrent oral ulceration (1 point): recurrent and multiple minor aphthous, major aphthous, or herpetiform ulcerations.

Recurrent genital ulceration (2 points): aphthous ulceration or scarring.

Eye lesions (2 points): anterior uveitis, posterior uveitis, or cells in vitreous on slit lamp examination; or retinal vasculitis.

Skin lesions (1 point): pseudofolliculitis, papulopustular lesions, erythema nodosum-like lesions, pyoderma gangrenosum-like lesions.

Vascular lesions (1 point): phlebitis, large vein thrombosis, aneurysm, arterial thrombosis.

Positive pathergy test (1 point): 2 mm erythema 24 to 48 hours after a #20- to 21-gauge needle prick to a depth of 5 mm.

Arthritis associated with Behçet’s disease:

Approximately 50% of patients will develop signs or symptoms of joint involvement. The arthritis is usually migratory, monoarticular or oligoarticular, and asymmetric, principally affecting the knees, ankles, elbows, and wrists. Arthritic flares typically last for several weeks. Enthesopathy is common especially in patients with acneiform lesions. Shoulders, spine, sacroiliac joints, hips, and small joints of the hands and feet are infrequently involved and should suggest another disease (e.g., HLA-B27-associated arthropathy). The arthritis may be polyarticular and occasionally resembles rheumatoid arthritis. Erosive changes are rare. Synovial fluid cell counts average 5000 to 10,000/mm3, and neutrophils predominate. Note that arthralgia is more common in Behçet’s disease but lacks diagnostic value.

International Criteria for BS - point score

System: Scoring 4 indicates Behcet’s diagnosis
Sign/symptom	Points
Ocular lesions	2
Genital aphthosis	2
Oral aphthosis	2
Skin lesions	1
Neurologic manifestations	1
Vascular manifestations	1
Vascular manifestations	1
Positive pathergy test*	1