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behcet_disease [2026/02/04 21:24] – created Scott Larsonbehcet_disease [2026/02/04 22:46] (current) – [Etiology] Scott Larson
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 ====== Behçet's Disease ====== ====== Behçet's Disease ======
  
 +====Main Features====
 +  *  Systemic Vasculitis characterized by recurrent oral ulcers (nearly all) with variable involvement of skin, eyes, joints, vascular system, GI tract and CNS
 +
 +====Eye Findings====
 +  * Occur in about 50% and if present is usually bilateral 
 +  * Uveitis 
 +    * Anterior with hypopyon, isolated anterior only about 10%
 +    * Vitritis
 +    * Retinitis
 +    * Occlusive retinal vasculitis 
 +    * Panuveitis 
 +====Other Findings====
 +  * Skin, Mucosa and articular
 +    * Oral ulcers 98%
 +    * Genital ulcers 60-65%
 +    * Skin lesions 75%
 +      * pseudofoliculitis
 +      * papulopustular lesions (acne-like)
 +      * erytherma nodosum
 +    * Arthralgia or arthritis 50%
 +      * knees, ankles, wrists, and elbows
 +      * enthesopathy 
 +  * CNS 10-30%
 +    * Mesodiencephalic and brainstem inflammation 
 +    * Encephalitis
 +    * Meningitis
 +    * Myelitis
 +    * Neurocognitive dysfunction
 +    * Cerebral-vein thrombosis
 +  * Cardiovascular
 +    * Arterial 2-18%
 +      * Artery aneurisms
 +      * arterial stenosis or thrombosis
 +    * Heart 6%
 +      * Valvulitis
 +      * Myopericarditis
 +      * Coronary arteritis 
 +    * Venous 15-40%
 +      * Deep vein thrombosis of limbs, pulmonary, suprahepatic
 +      * Superficial thrombophlebitis 
 +  * GI 0-20%
 +    * Mucoasal ulcers
 +    * Abdominal pain
 +    * Hemorrhage and/or perforation 
 +====Etiology====
 +  * Genetically predisposed are exposed to environmental triggers leading to activation of cellular signally pathways leading to tissue inflammation and damage. 
 +  * HLA B*51 increases the risk by 6
 +  * Highest prevalence in Turkey: 420 cases per 100,000
 +  * US: 5.2 cases per 100,000 
 +====Resources====
 +  * [[https://www.nejm.org/doi/full/10.1056/NEJMra2305712?utm_source=openevidence|Behçet's Syndrome. Saadoun D et al. N Engl J Med 2024;390:640-651]]
 +  * [[https://pmc.ncbi.nlm.nih.gov/articles/PMC10253549/|Behçet's Disease Uveitis. Joubert M, et al. J Clin Med. 2023;12(11):3648]]
 +
 +{{tag>syndrome}}