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behcet_disease [2026/02/04 22:27] Scott Larsonbehcet_disease [2026/02/04 22:46] (current) – [Etiology] Scott Larson
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 ====== Behçet's Disease ====== ====== Behçet's Disease ======
 +
 ====Main Features==== ====Main Features====
   *  Systemic Vasculitis characterized by recurrent oral ulcers (nearly all) with variable involvement of skin, eyes, joints, vascular system, GI tract and CNS   *  Systemic Vasculitis characterized by recurrent oral ulcers (nearly all) with variable involvement of skin, eyes, joints, vascular system, GI tract and CNS
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     * Panuveitis      * Panuveitis 
 ====Other Findings==== ====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==== ====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==== ====Resources====
-  * [[link|Title]]+  * [[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}} {{tag>syndrome}}