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      Behçet’s Disease – Case Presentation and Review Literature

      case-report

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          Abstract

          BACKGROUND:

          Behçet’s syndrome is associated with inflammation of various areas of the body. Sy. Behcet is a rare, chronic, recurrent disease characterised by changes in the: Arteries that supply blood to the body’s tissues, veins that take the blood back to the lungs, the back of the eye’s retina, brain, joints, skin and bowels. There is a close correlation between the geographical distribution of HLA-B51 and its prevalence. In the etiopathogenesis, there are indications of genetic susceptibility associated with environmental influence. Although aetiology is not yet known, it is thought of viral or autoimmune genes but is not yet confirmed by relevant analysis.

          CASE REPORT:

          This was a case of a 29 years old young female presenting with recurrent oral and genital ulcers. Eye lesions usually start in one eye and then pass to the other eye. They are like iridocyclitis extending very quickly to another eye. Three months later, a few shifts were introduced in the form of small initial ulcers, which for 4-5 days have been enlarged and then epithelized by leaving the catapult in the genital mucus. In the skin of the lower extremities, papules appear to be as large as corn grain.

          CONCLUSION:

          Diagnosis of Behçet’s syndrome is determined based on eye changes, oral mucous and genital mucosa. Treatment of Behçet’s syndrome depends on the severity and the location of its manifestations.

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          Most cited references9

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          Cerebral venous thrombosis in Behçet's disease.

          To analyze the clinical findings, treatment, outcome, and prevalence of cerebral venous thrombosis (CVT) in a large cohort of patients with Behçet's disease (BD) from a single center. We reported a series of 64 consecutive patients with CVT who fulfilled the international criteria for BD. Multivariate analysis was performed to define factors that affect prognosis. Among a cohort of 820 patients with BD, CVT was present in 64 (7.8%). Compared with BD patients without CVT, those with CVT had lower parenchymal central nervous system involvement (4.7% versus 28.7%; P = 0.0001) and higher extraneurologic vascular lesions (62.5% versus 38.8%; P = 0.03). Up to 90% of patients responded to anticoagulation therapy without severe hemorrhagic complications. Neither steroid nor immunosuppressant use provided better outcome. Severe visual loss due to optic atrophy was the main complication of CVT, being found in 15% of patients. In multivariate analysis, papilledema (odds ratio [OR] 7.1, 95% confidence interval [95% CI] 1.6-31.9) and concurrent prothrombotic risk factors (OR 4.6, 95% CI 1.1-20.2) were independently associated with the occurrence of sequelae. Factors associated with relapse of thrombosis were concurrent prothrombotic risk factors (hazard ratio [HR] 4.9, 95% CI 1.5-15.4) and a peripheral venous thrombosis (HR 2.8, 95% CI 0.7-10.5). After a mean +/- SD followup of 8.2 +/- 6.9 years, 4 deaths unrelated to CVT were noted. CVT in patients with BD may result in serious neurologic outcomes. Anticoagulation represents a safe and effective therapy. Extensive investigation of prothrombotic disorders should be considered.
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            Behçet's disease: review with emphasis on dermatological aspects*

            Behçet's disease is a systemic vasculitis characterized by attacks of acute inflammation, which can affect almost every vascularized area of the body. There is a close correlation between the geographical distribution of HLA-B51 and its prevalence. In the etiopathogenesis there are indications of genetic susceptibility associated with environmental influence. Among the involved genes are those that encompass innate and adaptive immunities. Polymorphisms and epistatic interactions in several genes are described, as well as the presence of imbalance lineage between HLA-B51 and A (MICA). Herpes simplex and Streptococcus sanguinis may be important extrinsic factors. An increase of Th1 response and of IL-21 is observed. The production of IL-21 is positively related to Th17 cells and negatively to T-regs. The mucocutaneous manifestations are Behcet´s disease markers, and their earlier onset indicates a worse prognosis. Recurrent oral ulcers have varied sizes and arrangements, genital ulcers are recurrent, leaving scars, skin lesions are multivaried, and pathergy, although not so frequent, is important for the diagnosis. There are numerous attempts to validate indexes that can evaluate the disease activity and among them the Mucocutaneous Activity Index. This is a specific score that can help with therapeutic decisions and to reduce morbidity, but still lacks validation. The clinical manifestations of other organs are described as well as treatment options.
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              Childhood-onset uveitis in Behçet disease:a descriptive study of 36 cases.

              To describe the demographic and clinical features, complications, treatment, and visual results in patients with childhood-onset Behçet uveitis. Observational case series. A retrospective study was made of 36 consecutive patients with Behçet disease seen at the Uveitis Service, Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, between January 1975 and January 2002. Inclusion criteria were fulfillment of the classification criteria of the International Study Group for Behçet Disease and onset of uveitis at 16 years of age or younger. The medical records of 36 patients with childhood-onset Behçet uveitis were reviewed. The main outcome measures were sex, age at onset of uveitis, the initial symptom of Behçet disease, clinical ocular features, ocular complications, systemic treatment, complications of treatment, and final visual acuity. Twenty-five patients were male, 11 were female. Mean age at onset of uveitis was 13.6 years. The initial symptom was oral ulcer in 63.8% of the patients. The majority of patients (83.3%) had bilateral involvement. Panuveitis was the most common form (86.2%). Retinal vasculitis and retinitis were the most common ocular findings seen in 83.3% and 68.2% of the involved eyes, respectively. Cataract, maculopathy, and optic atrophy were the most common complications seen in 46.9%, 45.4%, and 39.4% of the involved eyes, respectively. Immunosuppressive therapy was administered to 75% of the patients. Response to treatment was variable. The most common complications of systemic treatment were associated with corticosteroid therapy. Final visual acuity was worse than 0.1 in 22.7% of the involved eyes. Childhood-onset Behçet uveitis was more common among males. Bilateral panuveitis with retinal vasculitis and retinitis was the most common form of ocular involvement, similar to the adult patient. The treatment is challenging, as the use oral corticosteroids is associated with significant complications and the response to conventional immunosuppressive therapy is variable.
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                Author and article information

                Journal
                Open Access Maced J Med Sci
                Open Access Maced J Med Sci
                Open Access Macedonian Journal of Medical Sciences
                Republic of Macedonia (ID Design 2012/DOOEL Skopje )
                1857-9655
                25 October 2018
                03 October 2018
                : 6
                : 10
                : 1871-1874
                Affiliations
                [1 ]Family Medical Center of Prishtina, Prishtina, Kosovo
                [2 ]University Clinical Center of Kosovo, Clinic of Dermatology, Prishtina, Kosovo
                Author notes
                [* ] Correspondence: Mybera Ferizi. University Clinical Center of Kosovo, Clinic of Dermatology, Prishtina, Kosovo. E-mail: myberaf@ 123456hotmail.com
                Article
                OAMJMS-6-1871
                10.3889/oamjms.2018.393
                6236042
                fe7db01d-0a3e-40c3-9637-90895135becb
                Copyright: © 2018 Mergita Ferizi, Antigona Gerqari, Mybera Ferizi.

                This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).

                History
                : 04 July 2018
                : 06 September 2018
                : 07 September 2018
                Categories
                Case Report

                aft syndrome,behçet’s syndrome,mucosal ulceration,hla-b51

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