Behçet's Syndrome
Systemic disease associated with ...

Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
 • 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
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
 • 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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Contributed by

Dr. Ravi Singh K
Academic Hospitalist and APD @SinaiBmoreIMRes, Clinical reasoning,Simulation and POCUS enthusiast -
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