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