Behcet Disease - Dermatology - Lecture Slides, Slides of Dermatology

Download Behcet Disease - Dermatology - Lecture Slides and more Slides Dermatology in PDF only on Docsity! Behcet’s Disease Docsity.com Behcet’s: Epidemiology • Disease prevalence and expression vary geographically and it affects people of Middle Eastern or Far Eastern ancestry more often than those from other regions. • Turkey has the highest prevalence: 80 to 370 cases per 100,000 population. Docsity.com Behcet’s: Pathophysiology • The prevalence of the HLA-B51 allele is high among patients with Behçet's disease who are of Middle Eastern or Far Eastern ancestry (up to 81 percent of Asian patients have the allele) but not among white patients who live in Western countries (13 percent). • Studies suggest a possible pathogenic role of certain bacterial/viral antigens that have cross-reactivity with human peptides. Docsity.com Behcet’s: Pathophysiology • Endothelial activation in affected blood vessels has been proposed as a mediator of vascular inflammation as well as thrombosis in Behcet's. • Hyperfunction of neutrophils noted within active lesions Docsity.com Clinical Manifestations: Oral Lesions • Oral ulceration is usually an initial symptom and is seen in all patients at some time in the clinical course, sometimes precedes other manifestations by a number of years. • Oral aphthae which are grossly and histologically similar to common oral ulcers, but tend to be more extensive and often multiple • Lesions heal within about 10 days without scarring. Docsity.com Clinical Manifestations: Cutaneous Lesions • Pathergy refers to an erythematous papular or pustular response to local skin injury. • Defined as a greater than 5 mm lesion that appears 24 to 48 hours after skin prick by a needle. • Less common in European decent patients with Behcet's disease (10 to 20 percent), than in Eastern patients (50 to 75 percent). • The pathergy test can also be positive in Sweet's syndrome and pyoderma gangrenosum. Docsity.com Clinical Manifestations: Arthritis • Nonerosive, asymmetric, usually nondeforming arthritis occurs in about one- half of patients with Behcet's disease, particularly during exacerbations. • Most commonly affects the medium and large joints, including the knee, ankle, and wrist. Docsity.com Clinical Manifestations: Ocular • Ocular disease occurs in 25 to 75 percent of patients with Behcet's disease, and may progress to blindness. • Symptoms, including blurred vision, eye pain, photophobia, lacrimation, floaters • Uveitis is often the dominant feature of Behcet's disease. It is typically bilateral and episodic. Docsity.com Clinical Manifestations: Gastrointestinal • Symptoms include abdominal pain, diarrhea, melena, and sometimes perforation. • Gastrointestinal ulcerations occur most often in the terminal ileum, cecum, and ascending colon. • Histologically, the intestinal ulcers of Behçet's disease are indistinguishable from those of ulcerative colitis. • It is often difficult to distinguish between Behçet's disease and inflammatory bowel diseases, because of the similarity in extraintestinal symptoms Docsity.com Clinical Manifestations: Gastrointestinal • The other parts of the gastrointestinal system and liver (except in Budd-Chiari syndrome), pancreas, and spleen are rarely involved. • A large Japanese autopsy registry of subjects with Behcet’s, reported 5 cases of histologic pancreatitis in a total 170 cases. Docsity.com Clinical Manifestations: Neurologic • Neurologic disease occurs in fewer than one-fifth of patients with Behcet's disease, more frequently in men than women. • Classically, meningitis or meningoencephalitis, neurologic deficits such as motor disturbances and brain-stem symptoms, and psychiatric symptoms including personality changes develop more than five years after Behçet's disease is diagnosed. Docsity.com Clinical Manifestations: Vascular • This may lead to stenosis or aneurysm formation. Rupture of such aneurysms may be fatal. • Vascular lesions in the lung, including thrombosis, aneurysm, and arteriobronchial fistula, cause recurrent episodes of dyspnea, cough, chest pain, and hemoptysis. Docsity.com Vascular disease in 728 patients with Behcet's disease Number of patients Venous disease Deep venous thrombosis 221 Subcutaneous thrombophlebitis 205 SVC occlusion 122 IVC occlusion 93 Cerebral sinus thrombosis 30 Budd-Chiari syndrome 17 Other venous occlusion* 24 Arterial disease Pulmonary artery occlusion or aneurysm 36 Aortic aneurysm 17 Extremity arterial occlusion or aneurysm 45 Other arterial occlusion or aneurysm 42 Right ventricular thrombus 2 Docsity.com Behcet’s Disease: Diagnosis • There are no pathognomonic symptoms or laboratory findings, the diagnosis is made on the basis of clinical findings • New international criteria were published in 1990 Docsity.com Behcet’s Disease (Mucocutaneous): Treatment • Treated with topical anesthetics, high potency glucocorticoids, intralesional steroids, colchicine. • Ulcers that are refractory to these treatments, require systemic steroids, thalidomide, or the immunosuppressive agents. Docsity.com Behcet’s Disease (Ocular): Treatment • Ocular lesions are treated with topical mydriatics, glucocorticoids. • Relapses and/or progressive disease may occur with steroid treatment alone. • Combination therapy, as for systemic disease, is often necessary. • There is evidence of benefit from cyclosporine and azathioprine. No efficacy for cyclophosphamide or colchicine. Docsity.com Behcet’s Disease (Neurologic): Treatment • Aseptic acute meningitis or meningoencephalitis in the early phase of the disease responds well to treatment with corticosteroids. • In contrast, chronic progressive central nervous system disease is resistant to all the currently available therapies. Docsity.com Behcet’s Disease: Prognosis • Behcet's disease has an undulating course of exacerbations and remissions, and may become less severe after approximately 20 years. • The disease appears to be more severe in young, male, and Middle Eastern or Far Eastern patients. Docsity.com Behcet’s Disease: Prognosis • Mucocutaneous, articular and ocular disease are often at their worst in the early years of disease. • Central nervous system and large vessel disease, if they develop, typically do so later in the disease course. Docsity.com Behcet’s Disease: Prognosis • In one study, 20 percent of patients with chronic neurologic involvement died within seven years. • Half of patients die within three years after the onset of hemoptysis. Docsity.com