scholarly journals Occurrence of recurrent stomatitis, watery diarrhea and erythema nodosum in a patient with 20 year history of Takayasu's disease.Coexistence of Takayasu's disease and incomplete BehCet's disease ?

1994 ◽  
Vol 17 (2) ◽  
pp. 99-105
Author(s):  
Satomi Nakano ◽  
Kensei Tsuzaka ◽  
Masashi Akizuki ◽  
Norifumi Hibi ◽  
Makio Mukai
2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Briosa ◽  
A R Almeida ◽  
A C Gomes ◽  
A R Pereira ◽  
A Marques ◽  
...  

Abstract Introduction Intracardiac masses are always a challenging diagnosis, especially when it involves the right side of the heart. There are multiples etiologies that can be responsible for these masses, namely thrombosis, neoplasm or vegetations. Occasionally, these may be related to an autoimmune process not yet discovered. Case Report 17-year-old male, with a previous history of genital ulcers, medicated with penicillin with complete resolution of symptoms. In January 2019, he started an history of recurrent fever, associated with right anterior thoracalgia, weight loss and oral afthosis. He went to the emergency department several times, where he was medicated with antibiotic, with partial symptom relief. Three months later, he returned to medical attention due to an episode of abundant hemoptysis, followed by hematemesis and cough. At hospital admission, he was hemodynamically stable, tachycardic (100/min) and with occasional episodes of cough. Cardiac and pulmonary auscultation were unremarkable. Thoracic CT revealed the presence of pulmonary thromboembolism (PTE) and a large mass in the right ventricle (RV). It was performed an echocardiogram (echo) that confirmed the presence of a large mass in the RV (50x53mm) from which a projecting hypermobile mass appeared to prolapse into the right atrium. Taking into account the diagnosis of PTE and the presence of a right ventricular mass, the patient was hospitalized and started anticoagulation. The case was immediately discussed with cardiac surgery, that confirmed that there was no surgical indication. During hospitalization, there were no more episodes of hemoptysis or hematemesis.Consecutive echos were performed, that did not reveal a significant decrease in mass dimensions despite anticoagulation. Viral serologies and autoimmunity panel were all negative. Cardiac RMI was performed raising the suspicion of a possible mass covered with thrombus. After discussion with rheumatology, and according to clinical signs, the hypothesis of vasculitis was placed, and the patient started treatment with steroids. This treatment had to be suspended after a few days due to an infectious intercurrence. After a course of antibiotic therapy, the patient started therapy with cyclophosphamide with good clinical and echocardiographic response (reduced mass dimensions). It was admitted Behçet’s disease with cardiac complications, and the patient was referred to the rheumatology consultation. Conclusion Behçet’s disease is a multi-system, chronic disorder that behaves like vasculitis.There are some typical clinical manifestations associated with this disease, such as oral and genital afthosis, uveitis, arthritis, skin lesions and nervous system involvement.Presentations with cardiac symptoms are one of the extremely rare manifestations of this disease, posing a challenge for the treating physician. Abstract 475 Figure. Right ventricular mass


2012 ◽  
Vol 23 (9) ◽  
pp. 683-684
Author(s):  
S Patel ◽  
K Prime

This report describes a young black Caribbean woman with a three-year history of recurrent genital ulceration initially diagnosed as genital herpes. This diagnosis had been made clinically by the general practitioner but never confirmed on culture or nucleic acid amplification testing; sequential treatment with aciclovir and famciclovir over a four-month period did not alleviate her genital symptoms. Presentation to our genitourinary (GU) medicine clinic identified painful aphthous genital and oral ulcers, erythema nodosum bilaterally and a history of eye irritation. A clinical diagnosis of Behçet's disease was made. Treatment with hydrocortisone mouth pellets and dermovate ointment was initiated in addition to dermatology and ophthalmology referral. This report reminds clinicians to consider alternative diagnoses in clinical practice when faced with patients who are resistant to treatment. It also highlights the importance of recognizing systemic disease and maintaining a holistic approach when treating patients.


2016 ◽  
Vol 7 (1) ◽  
pp. 141-147 ◽  
Author(s):  
Roy Schwartz ◽  
Sara Borok ◽  
Michaella Goldstein ◽  
Anat Kesler ◽  
Keren Regev ◽  
...  

Purpose: To report a unique case of Behçet's disease that presented with atypical ocular manifestations. Methods: Case report. Results: A 23-year-old homosexual male presented with bilateral anterior uveitis, vitritis, neuroretinitis and a unilateral superior hemivein occlusion with frosted branch angiitis pattern. These were accompanied by systemic findings of recurrent oral aphthous ulcers, erythema nodosum, and neurological and gastrointestinal involvement. A positive HLA-B51 examination supported the diagnosis of Behçet's disease. Conclusion: Neuroretinitis and frosted branch angiitis may be the clinical manifestations of Behçet's disease and may present simultaneously.


Author(s):  
Sara Mendonça Freitas ◽  
Joana Silva Marques ◽  
Ana Grilo ◽  
Rodolfo Gomes ◽  
Fernando Martos Gonçalves

Behçet’s disease (BD) is a systemic vasculitis characterized by recurrent orogenital ulceration and several systemic manifestations (such as gastrointestinal involvement, vascular disease or arthritis). The pathogenesis is still unknown but the trigger role of certain pathogens such as Mycobacterium tuberculosis is well documented. Furthermore, patients with BD are more susceptible to tuberculosis due to immunity defects. Here, we describe the case of a 70-year-old woman with a history of recurrent oral aphthae and inflammatory arthritis presenting with extensive thrombosis of left upper limb major veins, a positive HLA B51 genotype and colon ulceration; hence, BD diagnosis was made after excluding other causes. Simultaneously, the patient had cutaneous abscesses not associated with immunosuppressive therapy with continuous development, and after recurrent negative tuberculosis work-up, M. tuberculosis was isolated in an abscess culture.


Author(s):  
З.C. Алекберова ◽  
Т.А. Лисицына ◽  
Р.Г. Голоева ◽  
А.В. Волков ◽  
Р.А. Осипянц ◽  
...  

Введение. Болезнь Бехчета (ББ) – системный васкулит неизвестной этиологии, характеризующийся рецидивами язвенного процесса в ротовой полости и на гениталиях, частым поражением глаз и рядом проявлений, включающих патологию суставов, желудочно-кишечного тракта, нервной системы и сосудов. Цель исследования: определить частоту и локализацию сосудистых проявлений у больных с ББ, уточнить связь последних с полом, возрастом и другой клинической симптоматикой ББ. Материалы и методы. Обследовано 250 больных ББ, сосудистая патология выявлена у 63 (25,2%) пациентов. Возраст больных с сосудистыми проявлениями составил 34,8 ± 10,4 лет, соотношение мужчины:женщины – 3,5:1. Сосуды вовлекались в процесс чаще в течение первых трех лет болезни. Поражалось в основном венозное русло, доля больных с артериальной патологией составила 9,5%. Результаты. У пациентов с сосудистой патологией по сравнению с больными без тромбозов достоверно чаще выявлялись узловатая эритема (71% против 44%; р = 0,002) и эпидидимит (22,4 % против 10,9%; р = 0,04), а генерализованный увеит (17,4% против 32%; р = 0,002) и ангиит сетчатки (22% против 40,6%; р = 0,008) встречались достоверно реже. В системе коагуляции у больных ББ с и без сосудистой патологии достоверных различий не выявлено. Заключение. Сосудистые нарушения выявлены у ¼ больных (преимущественно мужчин молодого возраста), развивались чаще в первые три года болезни и ассоциировались с узловатой эритемой, эпидидимитом и положительным тестом патергии. Introduction. Behcet's disease (BB) is a systemic vasculitis of unknown etiology; it is characterized by recurrent aphthous stomatitis, genital ulcers, uveitis and other manifestations including arthritis, pathology of gastrointestinal tract, nervous system and blood vessels. The aim of study: to determine frequency and localization of vascular manifestations in patients with BB, to clarify the relationship of manifestations with sex, age and other clinical symptoms of BB. Materials and methods. We examined 250 patients with BB, vascular pathology was revealed in 63 (25,2%) patients. The age of patients with vascular manifestations was 34,8 ± 10,4 years, the ratio of men:women was 3,5:1. Vessels were involved in the process more often during the first three years of the disease. Mainly veins were affected, arterial pathology had 9,5% of patients. Results. In patients with vascular pathology erythema nodosum (71% vs. 44%, p = 0,002) and epididymitis (22,4% vs. 10.9%; p = 0,04) were significantly more frequent than in patients without thromboses, and panuveitis (17,4% vs. 32%; p = 0,002) and retinal vasculitis (22% vs. 40,6%; p = 0,008) were found significantly more rarely. There were no significant differences in coagulation system between patients with and without vascular pathology. Conclusion. Vascular disorders were detected in ¼ of patients (predominantly in men of young age), developed more often in the first three years of the disease and associated with erythema nodosum, epididymitis and a positive pathergy test.


Author(s):  
Aanchal Mehta ◽  
Julien Al Shakarchi ◽  
Lewis Meecham ◽  
Santhosh Vijay ◽  
Richard Downing

AbstractBehcet's disease (BD) is a rare multiorgan systemic disorder characterized by recurrent episodes of acute inflammation. Involvement of the vascular system, gastrointestinal tract, and central nervous system portends a poor prognosis.We report the case of a 54-year-old man who presented with a 2-week history of symptoms attributable to infrarenal aortic and left tibioperoneal trunk pseudoaneurysms, defined by CT angiography which also revealed right lower lobe pulmonary artery (PA) and right anterior tibial arterial aneurysms. A prior history of recurrent oral ulceration, periodic fever, cerebral venous sinus thrombosis, and aseptic endocarditis with pulmonary emboli invoked a diagnosis of BD. Immunosuppression was commenced immediately, following synchronous endovascular and open arterial intervention, except the PA aneurysm.He developed a fever and cough 8 days postoperatively as a consequence of COVID-19 infection from which he recovered without complications.The management of the patient's four aneurysms in different vascular territories and postoperative COVID-19 infection in the presence of mandatory immunosuppression are discussed.


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