Pathophysiology
systemic vasculitis of both large and small vessels
Signs and Symptoms
1) classic triad – painful oral aphthous ulcers, genital ulcers, inflammation of the eye (hypopyon uveitis, retinal vessel thrombosis, blindness) 2) inflammation of heart and CNS 3) acne-like skin lesions 4) venous thrombosis 5) aneurysm formation 6) GI hemorrhage 7) arthritis in ankles and knees
Characteristic Test Findings
Laboratory – 1) autoantibodies to oral mucosa basement membrane in 50% 2) leuckocytosis 3) increased sed rate 4) C-reactive protein increased
Histology/Gross Pathology
1) vessel walls infiltrated by lymphocytes and plasma cells 2) ulcers in mouth and genitals have a yellowish central necrotic base
Associated Conditions
1) HLA-B51 2) blindness 3) obstruction of superior vena cava with thrombus 4) esophagitis 5) intracranial hypertension 6) psychiatric problems 7) multiple sclerosis-like syndrome
Inheritance/Epidemiology
1) most common in Japan 2) occurs in 1/10,000 3) young males most commonly affected
Treatment
1) corticosteroids 2) thalidomide 3) aspirin 4) colchicine 5) alpha-INF 6) azathioprine
Tips for USMLE
hypopyon uveitis (acculumlation of pus in anterior chamber of eye) is almost pathognomonic for Behcet’s syndrome
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