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Behcet's Disease
Behcet's disease is a rare immune-mediated systemic vasculitis characterized by recurrent oral and genital ulcers. It was first described in 1937 by Turkish dermatologist Hulusi Behcet based on its hallmark triple-symptom complex of recurrent oral and genital ulcers and uveitis. Behcet's disease can affect multiple organ systems and commonly involves the eyes, skin, joints, and blood vessels. Diagnosis is based on clinical criteria including recurrent oral ulcers plus two of genital ulcers, eye lesions, skin lesions or a positive pathergy test. Treatment depends on symptoms but may include colchicine, corticosteroids, azathioprine, anti-T
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Behcet's Disease
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- 2. Behçet's disease (BEH-chets) •Also called Behçet's syndrome, Morbus Behçet, or Silk Road disease, is a rare immune-mediated systemic vasculitis. • Behçet's disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of –recurrent oral aphthous ulcers, –genital ulcers, and –uveitis
- 3. Behçet's disease (BEH-chets) •Also called Behçet's syndrome, Morbus Behçet, or Silk Road disease, is a rare immune-mediated systemic vasculitis. • Behçet's disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of –recurrent oral aphthous ulcers, –genital ulcers, and –uveitis
- 4. Behçet's disease (BEH-chets) •Also called Behçet's syndrome, Morbus Behçet, or Silk Road disease, is a rare immune-mediated systemic vasculitis. • Behçet's disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who first described the triple-symptom complex of –recurrent oral aphthous ulcers, –genital ulcers, and –uveitis
- 5. Behcet’s Syndrome Definition: Behçet's syndromeis a multisystem disorder presenting with- • Recurrent oral and genital ulcerations and • Ocular involvement • Diagnosis is clinical, based on internationally agreed diagnostic criteria
- 6. Behcet’s Syndrome Incidence, andPrevalence • Affects young males and females from the Mediterranean region, Middle East, and Far East, suggesting a link with the ancient Silk Route. • Males and females affected equally • Males often have more severe disease • Blacks are rarely affected
- 7. Behcet’s Syndrome Diagnostic Criteriaof Behçet's Disease • Recurrent oral ulceration plus two of the following: –Recurrent genital ulceration –Eye lesions –Skin lesions –Pathergy test
- 8. Behcet’s Syndrome: Pathogenesis Etiologyand pathogenesis - obscure Main lesions: • Systemic perivasculitis, early neutrophil infiltration • Endothelial swelling & panvasculitis • Vasculitis of vasa vasorum -formation of pseudoaneurysms
- 9. Behcet’s Syndrome: Pathogenesis Etiologyand pathogenesis - obscure Main lesion: • Panvasculitis Also seen: • Increased numbers of infiltrating CD4+ T cells • Circulating autoantibodies against – –Enolase of endothelial cells –Selenium binding protein –Anti-saccharomyces cerevisiae antibodies (also in Crohn's) • Association with HLA-B*51
- 10. Behcet’s- Clinical Features: 1.Orogenital ulceration • Recurrent aphthous ulcers are a sine qua non • Painful, shallow /deep with central necrotic base • Singly or in crops • Variable size • Located anywhere in the oral cavity • Persist X 1–2 weeks, subside without scars • The genital ulcers – • less common, but more specific • Painful • Spare the glans penis and urethra, and • produce scrotal scars
- 11. Behcet’s- Clinical Features: 2.Skin involvement -80% • Folliculitis • Erythema nodosum • An acne-like exanthem • Vasculitis (infrequent) • Sweet's syndrome • Pyoderma gangrenosum • Nonspecific skin inflammatory reactivity to any scratches or intradermal saline injection (pathergy test) is a common and specific manifestation.
- 12. Behcet’s- Clinical Features: 3.Eye involvement in 50% • Scarring and bilateral pan-uveitis • Iritis, posterior uveitis, • Retinal vessel occlusions, and • Optic neuritis
- 13. Behcet’s- Clinical Features: 4.Joints- 50% • Non-deforming arthritis or arthralgias • Knees and ankles
- 14. Behcet’s- Clinical Features: 5.Vascular Venous: 50% • Superficial/ deep peripheral vein thrombosis -in 30% • Pulmonary emboli –rare • SVC obstruction- occasional Arterial < 5% • Aortitis or peripheral arterial aneurysm • Arterial thrombosis • Pulmonary artery vasculitis (dyspnea, cough, chest pain, hemoptysis, and pulm infiltrates)- 5% • DD: Thromboembolic disease (it requires anti- inflammatory Rx not thrombolytic therapy
- 15. Behcet’s- Clinical Features: 6.Neurologic involvement (5–10%) • Brainstem involvement of serious prognosis (CNS-Behçet's syndrome). • Dural sinus thrombi (20%) (headache and increased ICP).
- 16. Behcet’s- Clinical Features: 6.Others • Gastrointestinal more in Japanese, resembles Crohn‘s • Epididymitis • Amyloidosis & glomerulonephritis - uncommon
- 17. Behcet’s-Investigations Mainly nonspecific indicesof inflammation • Leukocytosis • Elevated ESR • CRP
- 18. Behcet’s-Investigations Mainly nonspecific indicesof inflammation • Leukocytosis • Elevated ESR • CRP
- 19. Treatment: Behcet's Syndrome •The severity of the syndrome usually abates with time. Apart from patients with • CNS-Behçet's syndrome and • Major vessel disease, the life expectancy seems to be normal • The only other serious complication is blindness.
- 20. Treatment: Behcet’s Syndrome Mucousmembrane inv: • topical glucocorticoids • Serious cases- thalidomide Mucocutaneous manifestations and arthritis: • Colchicine Thrombophlebitis • Aspirin
- 21. Treatment: Behcet’s Syndrome Mucousmembrane inv: • topical glucocorticoids • Serious cases- thalidomide Mucocutaneous manifestations and arthritis: • Colchicine Thrombophlebitis • Aspirin
- 22. Treatment: Behcet’s Syndrome Uveitisand CNS-Behçet's • Systemic steroids • Azathioprine • Cyclosporin Pulmonary or peripheral arterial aneurysms • Cyclophosphamide • Anti– TNF