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 ◽  
...  

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.

2013 ◽  
Vol 34 (2) ◽  
pp. 321-327 ◽  
Author(s):  
Anton Lennikov ◽  
Zemfira Alekberova ◽  
Regina Goloeva ◽  
Nobuyoshi Kitaichi ◽  
Lev Denisov ◽  
...  

2015 ◽  
Vol 2015 ◽  
pp. 1-10 ◽  
Author(s):  
Cinzia Rotondo ◽  
Giuseppe Lopalco ◽  
Florenzo Iannone ◽  
Antonio Vitale ◽  
Rosaria Talarico ◽  
...  

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.


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

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.


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