Recurrent vulval ulceration: could it be Behçet's disease?

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.

2020 ◽  
Vol 8 ◽  
pp. 2050313X2098146
Author(s):  
Arti A Patel ◽  
Amelia Stutman ◽  
Prarak Patel ◽  
Madhavi Capoccia

Behcet’s disease is a chronic, idiopathic vasculitis with multisystem involvement commonly characterized by the classic triad of oral lesions, genital ulcerations, and uveitis. We discuss the case of a 22-year-old woman with a long-standing history of oral ulcers and vulvovaginal burning who presented with acute painless uveitis. With this presentation, there was an initial concern for infectious retinitis for which she was started on systemic antiviral therapy. Subsequent infectious disease workup was ultimately negative. Given her medical history and current presentation, she was diagnosed and treated for an acute inflammatory episode of ocular Behcet’s disease. The patient’s vision returned to baseline prior to discharge after treatment with systemic glucocorticoids. The diagnosis of Behcet’s disease in the setting of painless vision loss can oftentimes be elusive. However, it is important for clinicians to keep this condition as a differential diagnosis in patients presenting with acute onset uveitis as the progression of Behcet’s disease can lead to severe vision loss and blindness without prompt and adequate treatment.


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


2017 ◽  
Vol 4 (3) ◽  
pp. 62
Author(s):  
David T. Dulaney ◽  
Wassem Juakiem ◽  
Katherine Cebe ◽  
Angelo H. Paredes

Behcet’s disease (BD) is a multisystem mucocutaneous inflammatory condition characterized by recurrent genital and oral ulcers, ocular inflammation, and can involve the gastrointestinal tract. Treatment involves the usage of immunosuppressive agents to control the disease with glucocorticoids utilized for treatment of flares. Tumor necrosis factor inhibitors are frequently used to control the disease as well. We present the case of a 40 years old African American female presenting with intestinal BD that was refractory to adalimumab therapy. In conjunction with glucocorticoids, the patient’s intestinal disease was controlled with infliximab therapy. Currently, there have been no studies comparing the efficacy of TNFα inhibitors on the treatment of BD. Future studies are needed to compare the efficacy of TNFα inhibitor agents in the treatment of intestinal manifestations of BD.


2015 ◽  
Vol 14 (2) ◽  
pp. 193-196
Author(s):  
Ana Bittencourt Detanico ◽  
Marcelo Luiz Brandão ◽  
Ly de Freitas Fernandes ◽  
Carolina Parreira Ribeiro Camelo ◽  
Juliano Ricardo Santana dos Santos

Behcet's disease is form of systemic vasculitis of unknown etiology. One surprising feature is that arterial involvement is less common in this disease than venous forms, accounting for 1 to 7% of patients. In 7 to 30% of cases the vascular symptoms precede the clinical diagnosis of Behcet. We describe the case of a patient with Thromboangiitis Obliterans who was treated with lumbar sympathectomy and an aortobiiliac bypass. Around 1 year later he was diagnosed with Behcet's disease after presenting with oral ulcers, genital ulcers and scaling lesions on the hands.


2021 ◽  
Vol 12 (1) ◽  
pp. e72-e72
Author(s):  
Nasrin Zand ◽  
Leila Ataie-Fashtami ◽  
Parvin Mansouri ◽  
Mohsen Fateh ◽  
Afshan Shirkavand

Introduction: Behçet’s disease (BD) is a debilitating and chronic vasculitis which can affect multiple organs. Recurrent oral aphthosis is the most common clinical feature of BD. The remarkable pain associated with painful oral ulcers can exert a major impact on the patients’ quality of life. Relieving pain of these oral ulcers is an essential approach in the management of mucocutaneous lesions of BD. Non-thermal CO2 laser therapy (NTCLT) as a novel photobiomodulative approach has recently been used for significant and immediate pain reduction of some types of painful oral lesions such as recurrent aphthous stomatitis, pemphigus vulgaris, and so on. In this procedure by some considerations, the conventional CO2 laser is used as a non-surgical, non- thermal, photobiomodulative laser without any visible thermal adverse effects. In this article, we report our experiences about the analgesic effects of NTCLT on painful oral aphthous ulcers of BD. Case Presentation: We report four cases of BD, whose painful oral aphthous ulcers were irradiated with NTCLT. Immediately after NTCLT, the pain of the lesions was significantly relieved with no visible complications. Conclusion: The results of this study suggest that NTCLT could be proposed as a hopeful procedure for significant and instant pain relief of oral aphthous ulcers of BD without any visible thermal adverse effects.


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.


2021 ◽  
Vol 23 (5) ◽  
pp. 1079-1088
Author(s):  
E. S. Sorozhkina ◽  
G. I. Krichevskaya ◽  
N. V. Balatskaya ◽  
I. G. Kulikova ◽  
A. E. Andryushin ◽  
...  

Behcet's disease (BD) is a systemic disease underlyed by chronic vasculitis. Hyperactivity of innate and adaptive immunity plays important role in its pathogenesis. Uveitis occurs in 30-70% of the patients, often recurring and reducing visual function. The objective of our work was to study the features of systemic production of immune mediators in BD patients, depending on presence and activity of uveitis. 116 BD patients were divided into 3 groups: (1) 41 patients with active uveitis (UA), (2) 64 subjects with uveitis remission (UR), (3) 11 uveitis-free BD patients (WU). Control group (CG) comprised 34 conditionally healthy people. Detection rate (%) and contents (pg/ml) were measured for IL-1β IL-2, IL-4, IL-5, IL-6, IL-12p70, IL-13, IL-18, IFNγ, CCL2/MCP-1, CCL3/MIP-1α, CCL4/MIP-1β, CCL5/RANTES, CCL11/Eotaxin, СXCL1/GRO-α, CXCL8/IL-8, CXCL10/IP-10, CXCL12/SDF-1α, GM-CSF, TNFα in blood serum by means of multiplex analysis using MAGPIX analyzer (Luminex Corp., USA), Procarta Plex “Human Th1/Th2&Chemokine Panel 20 plex” kits (Bioscience, Austria). TGF-P1, TGF-P2 levels were assayed by ELISA-test (“Vfector-Best”). All the BD patients showed high detection rates of CXCL1/GRO-α (but not its level) in comparison with CG. Detection rate and levels of IL-6, IL-8 were increased in 1st and 2nd BD groups, compared to CG. In UR, unlike UA and WU groups, IL-4 was detected more often than in CG. WU patients showed increased detection rate of only CXCL1/GRO -α. When compared with UA, WU patients had lower serum concentrations of IFNγ, MCP-1, IP-10, MIP-1a, SDF-1α, TGF-β1; UR patients also showed decreased serum levels of IL-18, Eotaxin, GRO-α, RANTES, TGF-β2. Our results indicate the importance of angiogenic and proinflammatory chemokines and cytokines in pathogenesis of BD uveitis, as well as imbalanced production of various immunomediators. Higher detection rates and levels of IL-6 and IL-8 in UA and UR patients may result from weak persistent intraocular inflammation, even upon relief of clinical symptoms, thus, probably, requiring therapeutic correction.


Behçet’s disease (BD) is a chronic multisystem inflammatory disease characterized mainly by recurrent oral ulcers, ocular inflammation, genital ulcers, and skin lesions, presenting with remission and exacerbations. The pathogenesis of BD has not been fully elucidated since it has a broad spectrum of phenotypic heterogeneity and complex immunopathogenesis. BD has been considered that both environmental and genetic factors contribute to its onset and development. Recent advances in molecular biology and genetics show us that particular genes encompass both innate and adaptive immunity, and confirm the importance of the predominant polarization towards helper T cell (Th)1 versus Th2 cells, and the involvement of Th17 cells in BD.


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