scholarly journals Clinical and Pathological Manifestations with Differential Diagnosis in Behçet's Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-9 ◽  
Author(s):  
Aysin Kokturk
Keyword(s):  
Differential Diagnosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Laboratory Data ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Careful Evaluation ◽  
Behcet's Disease ◽  
Systemic Manifestations

Behçet's disease is a multisystemic inflammatory disease of unknown etiology which usually occurs as a trait of symptoms: aphthous stomatitis, genital ulcerations, and ocular disease. At the beginning of the disease the diagnosis is uncertain because of various clinical manifestations and a long period up to the full clinical picture manifestation. Since neither the laboratory data nor the histopathological signs are truly pathognomonic in Behçet's disease, the differential diagnosis depends on a careful evaluation of the medical history and meticulous physical examination to detect concomitant systemic manifestations. Sometimes, some laboratory test may help establish the diagnosis. Subspecialty referral to ophthalmology, rheumatology, neurology, and gastroenterology should be considered when indicated.

scholarly journals Pulmonary embolism in Behcet’s disease: a case report

2020 ◽  
Vol 90 (4) ◽  
Author(s):  
Despoina Moumtzi ◽  
Marianna Kakoura
Keyword(s):  
Pulmonary Embolism ◽  
Differential Diagnosis ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Rare Complication ◽  
Pulmonary Involvement ◽  
Unknown Etiology ◽  
Disease Exacerbation ◽  
Behcet's Disease

Behcet’s disease (BD) is a vasculitis of unknown etiology. It is often correlated with thrombophilic factors such as V Leiden. Pulmonary involvement is reported in 1-10% of patients. The most common manifestations are pulmonary aneurysms while pulmonary embolism is a rare complication. A 41-year old man with BD and V Leiden heterozygosity complained of pleurodynia and fever. Pleurodynia deteriorated in the following days and PE was confirmed by CT angiography, without the presence of aneurysms. After the exclusion of the antiphospholipid syndrome, a therapeutic dose of apixaban was initiated. Two weeks later, pleurodynia relapsed in combination with pleural effusion unilaterally. These findings were attributed to disease exacerbation. For this reason, we decided to enhance the immunosuppressive therapy. Six months later, CTPA showed complete remission of the clots. Vasculitis predisposes to thrombosis with or without coexisting thrombophilia. Clinicians should include them in their differential diagnosis and provide personalized treatment, based on immunosuppressants.

scholarly journals Mucocutaneous Involvement in Behçet’s Disease: How Systemic Treatment Has Changed in the Last Decades and Future Perspectives

2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Cinzia Rotondo ◽  
Giuseppe Lopalco ◽  
Florenzo Iannone ◽  
Antonio Vitale ◽  
Rosaria Talarico ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Current State ◽  
Life Threatening ◽  
Symptom Complex ◽  
Appropriate Therapy

Behçet’s disease (BD) is a multisystemic disorder of unknown etiology characterized by the “triple symptom complex” consisting of recurrent oral aphthosis, genital ulcers, and chronic relapsing bilateral uveitis. Recurrent mucocutaneous lesions are generally considered the hallmark of the disease, being the most common symptoms presenting at the onset of disease. Although the improvement of knowledge about the pathogenetic mechanism added important changes in the treatment management of BD clinical manifestations, thus avoiding the appearance of serious life-threatening complications which are disease related, the mucocutaneous lesions are still the most nagging clinical manifestations to be treated. In this work we reviewed the current state of knowledge regarding the therapeutic approaches for mucocutaneous lesions of BD mainly based on controlled studies to provide a rational framework for selecting the appropriate therapy for treating these troublesome features of the disease.

scholarly journals Atypical Vascular Involvement in a Case of Behçet's Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-5
Author(s):  
Alejandro Rodríguez Morata ◽  
Ana Hidalgo Conde ◽  
Carlos de la Cruz Cosme ◽  
Susana Gómez Ramírez ◽  
Rafael Gómez Medialdea
Keyword(s):  
Venous Thrombosis ◽  
Behçet’S Disease ◽  
Cerebral Venous Thrombosis ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Young Patients ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Vascular Intervention ◽  
Behcet's Disease

Introduction. Behçet's disease (BD) is a form of vasculitis of unknown etiology which is rare in our environment. It is characterized by a variety of clinical manifestations and usually affects young adults. Recurrent oral and genital ulcers are a characteristic and extremely frequent symptom, but mortality is linked with more significant symptoms such as aortic pseudoaneurysm, pulmonary pseudoaneurysm, and cerebral venous thrombosis.Patient and Method. We present a case of a young male with atypical BD and severe polyvascular involvement (previous cerebral venous thrombosis and current peripheral venous thrombosis, acute ischemia, and peripheral arterial pseudoaneurysm) who required urgent surgical intervention due to a symptomatic external iliac pseudoaneurysm.Result. The pseudoaneurysm was successfully treated, we performed an iliofemoral bypass, and we treated it with steroids and immunosuppressive therapy.Conclusions. These rare clinical manifestations highlight the importance of considering BD in young patients, even in usual cases of vascular intervention, whether arterial or venous in nature.

scholarly journals Epigenetics and Behçet’s Disease: DNA Methylation Specially Highlighted

2019 ◽  
Author(s):  
Jafar Farhadi ◽  
Mohammad Nouri ◽  
Ebrahim Sakhinia ◽  
Nasser Samadi ◽  
Zohreh Babaloo ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Methylation Status ◽  
Repetitive Sequences ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Epigenetic Factors ◽  
Behcet's Disease

Behçet's disease (BD) is a multisystem inflammatory disease with unknown etiology. Although evidence about the pathogenesis of BD is growing, the actual cause of this disease is unclear. Both genetic and epigenetic factors are claimed to play significant roles in BD. Epigenetic factors such as age, gender, smoking as well as exogenous factors like diet, infection, stress are related to the onset and clinical manifestations of BD. DNA methylation refers to a major epigenetic element which influences gene activities with catalyzing DNA using a set of DNA methyltransferases (Dnmts). DNA methylation status of many genes in patients with BD is different from that of healthy people. For example, cytoskeletal gene, Human Leukocyte Antigen (HLA) loci, Long interspersed nuclear element (LINE-1), and Arthrobacter luteus (Alu) repetitive sequences are different in the DNA methylation status in patients with BD and healthy controls. In this paper we reviewed, according to previous studies, the mechanisms of epigenetic, the epigenetic factors involved in the BD, and especially the effect of DNA methylation in the Behcet’s disease. Future studies are needed to identify the capability of specific DNA methylation alterations in BD in order to predict disease manifestations, medical course, and response to treatment.

scholarly journals Paediatric Behçet’s Disease: Data From A Single Center Experience in Turkey.

2021 ◽  
pp. 101-105
Author(s):  
Esra Bağlan ◽  
Semanur Özdel ◽  
Tülin Güngör ◽  
Deniz Karakaya ◽  
Evra Çelikkaya ◽  
...  
Keyword(s):  
Clinical Features ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Disease Onset ◽  
Clinical Manifestations ◽  
Unknown Etiology ◽  
Single Center ◽  
Behcet's Disease ◽  
Single Center Experience

Behçet’s disease (BD) is a multisystemic inflammatory disease with unknown etiology. It is characterized by recurrent oral and genital ulcerations, uveitis, and skin lesions, various musculoskeletal, gastrointestinal, central nervous system, and vascular manifestations. The aim of this study was to analyse the demographic characteristics and clinical features, treatment in Turkish paediatric BD from a single center experience. The records of 36 patients with BD who were diagnosed according to the International Study Group criteria between January 2017 and January 2019 in the department of paediatric rheumatology, were retrospectively reviewed. Data on demographic, clinical features and therapy were collected. A total of 36 (19 male) patients were included in this study. Mean age at disease onset was 9.36±4.45 years and mean age at diagnosis 13.99±2.83 years. The frequencies of signs/symptoms were: recurrent oral aphtosis 100%, genital ulcers 80.6%, musculoskeletal 30.6%, ocular 16.7%, neurological 11.1% and vascular involvement 11.1%, gastrointestinal 2.8%. Colchicine and corticosteroids were the main treatments. In this single-center retrospective study, we analyzed the data of paediatric BD and their treatment from a single center in Turkey. The presented small series and the literature review suggest that paediatric BD is a heterogeneous disease with varied clinical manifestations.

scholarly journals Gender Differences in Behçet’s Disease Associated Uveitis

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Didar Ucar-Comlekoglu ◽  
Austin Fox ◽  
H. Nida Sen
Keyword(s):  
Gender Differences ◽  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Vascular Involvement ◽  
Unknown Etiology ◽  
Behcet's Disease ◽  
Male Patients

Behçet’s disease is a systemic vasculitis of unknown etiology, characterized by oral and genital ulceration, skin lesions, and uveitis as well as vascular, central nervous system, and gastrointestinal system involvement. It is prevalent in the Middle East, Mediterranean, and Eastern Asia. The aim of this review is to evaluate the gender differences in clinical manifestations of Behçet’s disease, treatment responses, mortality, and morbidity. Behçet’s disease has been reported to be more prevalent in males from certain geographic regions and particular ethnic groups; however, recent reports indicate more even gender distribution across the world. There are gender differences in clinical manifestations and severity of the disease. Ocular manifestations, vascular involvement, and neurologic symptoms are more frequently reported in male patients whereas oral and genital ulcers, skin lesions, and arthritis occur more frequently in female patients. The disease can have a more severe course in males, and overall mortality rate is significantly higher among young male patients.

scholarly journals Behcet’s Disease: An In-depth Review About Pathogenesis, Gastrointestinal Manifestations And Management

2021 ◽  
Author(s):  
Anthony Nguyen ◽  
Shubhra Upadhyay ◽  
Muhammad Ali Javaid ◽  
Abdul Moiz Qureshi ◽  
Shahan Haseeb ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Age Groups ◽  
Clinical Manifestations ◽  
Signs And Symptoms ◽  
Vascular Disorder ◽  
Robust Algorithms ◽  
Behcet's Disease ◽  
Relapsing Remitting

Background: Behcet’s Disease (BD) is a complex inflammatory vascular disorder that follows a relapsing-remitting course with diverse clinical manifestations. The prevalence of the disease varies throughout the globe and targets different age groups. There are many variations of BD, however, intestinal BD is not only more common but has many signs and symptoms. Summary: BD is a relapsing-remitting inflammatory vascular disorder with multiple system involvement, affecting vessels of all types and sizes that targets young adults. The etiology of BD is unknown but many factors including genetic mechanisms, vascular changes, hypercoagulability and dysregulation of immune function are believed to be responsible. BD usually presents with signs and symptoms of ulcerative disease of the small intestine; endoscopy being consistent with the clinical manifestations. The mainstay of treatment depends upon the severity of the disease. Corticosteroids are recommended for severe forms of the disease and aminosalicylic acids are used in maintaining remission in mild to moderate forms of the disease. Key messages: In this review, we have tried to summarize in the present review the clinical manifestations, differential diagnoses and management of intestinal BD. Hopefully, this review will enable health policymakers to ponder over establishing clear endpoints for treatment, surveillance investigations and creating robust algorithms.

scholarly journals Immunopathogenesis of Ocular Behçet’s Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-13 ◽  
Author(s):  
Un Chul Park ◽  
Tae Wan Kim ◽  
Hyeong Gon Yu
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Retinal Vasculitis ◽  
Skin Lesions ◽  
Unknown Etiology ◽  
Ocular Involvement ◽  
Inflammatory Disorder ◽  
General Hypothesis ◽  
Behcet's Disease

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.

Mucocutaneous involvement in behçet’s disease

2020 ◽  
Vol 5 (5) ◽  
pp. 01-03
Author(s):  
Z. alaya ◽  
R amri ◽  
A. fraj ◽  
I. chaabane ◽  
W. Garbouj ◽  
...  
Keyword(s):  
Behçet’S Disease ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Chronic Inflammatory Disease ◽  
Behcet's Disease ◽  
Male Predominance ◽  
Skin Ulcers ◽  
Systemic Manifestations ◽  
Inflammatory Drugs ◽  
Perianal Ulcers

Behçet's disease is a chronic inflammatory disease characterized by its clinical polymorphism associating mucocutaneous involvement to systemic manifestations. The mucocutaneous lesions are considered the hallmark of the disease, being the most common symptoms presenting at the onset of disease. Our objective was to determine the characteristics of this skin involvement during Behçet's disease. We conducted a descriptive study over a period of 30 years, having collected all patients with Behçet's disease. These were 98 patients. A male predominance was observed in our studied population with a Sex Ratio of 2.5. The mean age at diagnosis was 34 years. Mucocutaneous involvement was observed in all patients. Oral aphthosis was constant and genital ulcers, were observed in 81 cases. The other mucocutaneous manifestations were: pseudofolliculitis (61 cases), erythema nodosum (7 cases), skin ulcers (4 cases), acneiform lesions (2 cases), perianal ulcers (1 case), skin ulceration (1 case) and erythema multiforme. (1 case). All of our patients were treated with colchicine. Corticosteroids and non-steroidal anti-inflammatory drugs were each indicated in one case for resistant forms.

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