Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young adults 20 to 40 years of age
 • Most common in Asia
 • HLA-B51 association
 • M > F
Clinical Manifestations:
 • Oral Ulcerations: Recurrent oral aphthous ulcerations (painful)
 • Urogenital Lesions (75% or more): Genital ulceration
 • Ocular Disease (25-75%): Inflammatory eye disease (panuveitis, retinal vasculitis)
 • Cutaneous Lesions (75%): acneiform lesions, erythema nodosum (septal panniculitis), superficial thrombophlebitis, pyoderma gangrenosum like lesions, pyoderma gangrenosum like lesions, erythema multiforme-like lesions, palpable purpura, Pathergy (an inflamed response to local skin injury)
 • Neurologic Disease: Lesions in the corticospinal tract, brainstem, periventricular white matter, spinal cord, and basal ganglia, CNS (brainstem lesions, aseptic meningitis), MCC CNS symptoms (headache and diplopia)
 • Vascular Disease: Arterial (MCC-small vessel vasculitis. Medium and large also involved), Venous disease (SVC/IVC occlusion, Budd-Chiari syndrome, dural sinus thrombosis, VTE)
 • Cardiac Disease: Pericarditis, myocarditis, coronary arteritis +/- myocardial infarction, coronary artery aneurysms
Diagnosis:
No pathognomonic laboratory tests in Behcet syndrome (Clinical Dx). At least three episodes of recurrent oral ulcers in 12 months plus at least two more of the following:
 • Genital ulcers 
 • Eye involvement (uveitis or retinal damage)
 • Skin lesions
 • Positive pathergy test (this is a skin prick test that can confirm Behcet disease)
Differential Diagnosis:
 • Genital ulcerations
 • Oral aphthae : SLE, Reactive arthritis,ervical adenopathy (PFAPA) syndrome, hyperimmunoglobulin D syndrome, and A20 haploinsufficiency (HA20)
 • Gastrointestinal : Crohn disease, ulcerative colitis, spondyloarthritis
 • Ocular: Giant retinal tears, Ischemia, Leukemia, Lymphoma Ocular melanoma
 • Arthritis: SLE, Reactive Arthritis, AS
Treatment:
 • Oral and genital ulcers-Topical glucocorticoids
 • Recurrent mucocutaneous lesions-Colchicine
 • Recurrent and chronic arthritis-Azathioprine, interferon alfa, or tumor necrosis factor CTNF)-a inhibitors
 • Uveitis- Ophthalmology collaboration
 • CNS exacerbations: high-dose glucocorticoids and azathioprine
 • Severe or refractory disease : monoclonal TNF-aIpha inhibitor + oral DMARD
 • Nonsurgical abdominal pain: Glucocorticoids 5-ASA or azathioprine

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