Behçet disease

Clinical Manifestations

Skin and mucosal
- Oral ulcers (98%), which are usually painful, recurrent, and can involve the soft and hard palate, buccal mucosa, tongue, gingiva, lips, and tonsils, and usually heal without scarring
- Genital ulcers (65%): recurrent, mostly heal with scarring, most commonly on scrotum in men and vulva or vagina in women
- Orchiepididymitis (6%)
- Skin lesion (75%), including pseudofolliculitis, acne-like papulopustular lesions, erythema nodosum, positive pathergy test
Joints (50%)
- Oligoarticular arthritis: primarily involving knees, ankles, wrists, and elbows; non-erosive and non-deforming, and more common in patients with acneiform skin lesions
- Can be a polyarticular arthritis as well
- Usually excludes sacroiliitis
- Enthesopathy
Ocular (50%): hypopyon, non-granulomatous panuveitis, retinal vasculitis
CNS involvement (10-30%): mesodiencephalic and brain-stem inflammation, encephalitis, meningitis, myelitis, neurocognitive dysfunction, cerebral-vein thrombosis
Cardiovascular
- Arterial (2-18%): arterial aneurysms, arterial stenosis, arterial thrombosis
- Cardiac (6%): valvulitis, myopericarditis, coronary arteritis
- Venous (15-40%): DVT, superficial thrombophlebitis
Gastrointestinal (0-20%): mucosal ulcers, abdominal pain, hemorrhage, perforation

Recurrent oral ulcerations (aphthous or herpetiform) at least three times in one year, plus
Any two of the following:
- Recurrent genital ulcerations
- Eye lesions 9uveitis or retinal vasculitis) observed by an ophthalmologist
- Skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions, aceiform nodules) in adult patients not treated with corticosteroids
- Positive pathergy test read by a physician at 24 to 48 hours
Sensitivity 95%, specificity 96%

Isolated skin and mucosal ulcers: tropical treatments for active ulcers, with colchicine as preventative treatment
Refractory or widespread lesions or ulcers: prednisone 15 mg daily for 1 week, followed by a taper over several weeks
Other options for more advanced presentations include systemic glucocorticoids, azathioprine, TNF-α inhibitors,