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Behçet's Disease is a syndrome characterized by recurrent, painful oral aphthous ulcers, in conjunction with 2 or more systemic manifestations – a common systemic manifestation of the disease being cutaneous lesions, which often present as papules that ulcerate to form ulcerations which exhibit the same pathergy phenomenon as pyoderma gangrenosum and are thus difficult to heal. We present a case of a type 2 diabetic male with previously undiagnosed Behçet's Disease who presented to clinic with an atypical lower extremity wound. Due to the atypical nature of the patient's wound, combined with information from the patient regarding the history of the wound and other illnesses he had, the wound was biopsied and the results from the biopsy combined with clinical suspicion yielded a diagnosis of Behçet's Disease. After understanding the nature of the wound, management of the ulceration was adjusted appropriately so as to properly treat the patient with both local wound care and medical management for the disease. Patient was referred to rheumatology for further workup and long-term management of Behçet's Disease. Correct identification and treatment of the systemic disease, as well as the lower extremity ulcerations, in patients with Behçet's Disease are critical to avoid organ and limb-threatening complications. Had the diagnosis of Behçet's not been made in this case, the patient's wound would have been at increased risk of disease-related complications – ranging from non-healing ulceration leading to amputation to renal failure to loss of eyesight. [ABSTRACT FROM AUTHOR]

Purpose: To report the pattern of uveitis in a major ophthalmology center in the central district of Thailand.Methods: A retrospective study was performed in uveitis cases visiting the Department of Ophthalmology at Rajavithi Hospital, Thailand, from January 2007 to October 2012.Results: Four hundred and forty-six patients (mean age 42 years, female 53.8%) were included in the study. Uveitis was unilateral in 51.1% of cases. Anterior uveitis was the most common (44.8%) case, closely followed by panuveitis (40%), posterior uveitis (14.3%), and intermediate uveitis (0.9%). Specific diagnosis was established in 51.6% of patients. The three most common specific diagnoses were Vogt-Koyanagi-Harada (VKH) disease (22.4%), followed by Behçet disease (6.7%) and herpetic anterior uveitis (5.8%).Conclusions: The most common type of noninfectious uveitis group was VKH, while herpetic anterior uveitis was the most common type of infectious uveitis in the central district of Thailand. [ABSTRACT FROM AUTHOR]

Infections are considered an environmental trigger for exacerbations of immune-mediated diseases. We aimed to establish if common viral infections could be identified as a precipitant of Behçet disease (BD) with or without neurological involvement and to assess the variability of the immune response to common viruses. We also investigated whether cytokines and chemokines could be markers of neurological involvement. Finally, we explored if anti-basal ganglia antibodies (ABGAs) would be associated with neurological involvement in BD. Our study included 14 individuals with BD with neurological involvement (neuroBehçet syndrome — NBS), 16 individuals with BD without neurological involvement and 18 healthy controls (HC). Overall we found a tendency for increased levels of anti-viral IgG antibody levels in BD, more evident in NBS patients versus HC. Epstein–Barr viral-capsid antigen IgG titres were increased in NBS patients versus other BD patients (p = 0.032). Anti-measles antibody titres induced by vaccination were similarly elevated. ABGAs were not detected in the serum of our cohort. Raised levels of serum IL-8 in some BD patients did not reflect clinical activity or severity. In conclusion, there was evidence for a polyclonal immune activation rather than a specific virus effect in the sera of individuals with BD or NBS. [ABSTRACT FROM AUTHOR]

Behçet's disease (BD) is a multisystem inflammatory disorder. Intracardiac thrombus formation (ICTF) is an uncommon but important complication of BD. To highlight recent insights into this disease, we aimed to review ICTF and other systemic involvements associated with ICTF in BD. We conducted a comprehensive review of the relevant literature in MEDLINE and EMBASE from 1966 to 2014 to analyze cumulated data about ICTF in BD. We aimed to evaluate 93 cases of BD with ICT (group 1), four of which have been recently identified and have not been discussed in the relevant literature yet, and to compare the frequency of pulmonary, venous and arterial involvements in group 1 and general Behçet population (group 2). The right heart was the most common site of ICTF in group 1. Pulmonary involvement, venous involvement (especially venous thrombosis) and arterial involvement were more frequent in group 1 than in group 2 (56 vs. 0.7 %, 42 vs. 10 % and 38 vs. 0.8 %, respectively, p < 0.0001). The diagnosis of BD should be considered if a patient presents with a mass in the right-sided cardiac chambers, even in the absence of the characteristic clinical manifestations of the illness. This approach is particularly applicable if the patient is a young man from the Mediterranean basin or the Middle East. All Behçet patients with ICTF must be investigated with thoracic computed tomography for pulmonary and arterial involvements and lower extremity venous Doppler ultrasonography for venous thrombosis, regardless of whether they are symptomatic for these systems. [ABSTRACT FROM AUTHOR]

Background and aims Behçet syndrome is a systemic inflammatory condition characterized by muco-cutaneous and ocular manifestations, with central nervous system, vascular and/or gastro-intestinal involvement. The association of microbiota with Behçet syndrome has not been shown yet. Our work was aimed to compare the gut microbiota structure and the profiles of short-chain fatty acids production in Behçet syndrome patients and healthy control relatives. Methods Here, we compared the fecal microbiota of 22 patients with Behçet syndrome and that of 16 healthy co-habiting controls, sharing the same diet and lifestyle by pyrosequencing of the V3–V4 hypervariable regions of the 16 rDNA gene and biochemical analyses. Results Our analyses showed significant differences in gut microbiota between Behçet patients and healthy cohabitants. In particular we found that Behçet's patients were significantly depleted in the genera Roseburia and Subdoligranulum . Roseburia showed a relative abundance value of 10.45 ± 6.01% in healthy relatives and 4.97 ± 5.09% in Behçet's patients, and Subdoligranulum , which reached a relative abundance of 3.28 ± 2.20% in healthy controls, was only at 1.93 ± 1.75% of abundance in Behçet's patients. Here we report, for the first time, that a peculiar dysbiosis of the gut microbiota is present in patients with Behçet syndrome and this corresponds to specific changes in microbiome profile. A significant decrease of butyrate production ( P = 0.0033) in Behçet's patients was demonstrated. Butyrate is able to promote differentiation of T-regulatory cells, and consequently the results obtained prompt us to speculate that a defect of butyrate production might lead to both reduced T-reg responses and activation of immuno-pathological T-effector responses. Conclusions Altogether, our results indicate that both a peculiar dysbiosis of the gut microbiota and a significant decrease of butyrate production are present in patients with Behçet syndrome. [ABSTRACT FROM AUTHOR]

Introduction: Behçet's disease (BD) is a rare vasculitis in sub-Saharan Africa. Vascular thrombosis, especially venous, is common in this condition and also constitutes a basic diagnostic criterion. Its affection of the superior vena cava is rather rare with only a few cases described in the literature. Case report: A 42-year-old male patient was seen at consultation presenting with a pulsatile, warm and slightly painful right latero-cervical swelling extending to the supraclavicular fossa with the presence of collateral venous circulation for three weeks prior to presentation associated with a mild headache. There were oral and genital ulcerations and erythematous skin lesions associated with a history of inflammatory recurrent arthralgia. Chest computed tomo-angiography showed cruoric internal jugular vein thrombosis extending to the superior vena cava with significant venous collateral circulation. The patient was treated with prednisolone (1 mg/kg/day) and colchicine (2 mg/day), as well as anticoagulation with heparin and vitamin K antagonist (Acenocoumarol) with regular INR monitoring. Clinical evolution was favorable during hospitalization, with residual discrete right supraclavicular swelling. There was no bleeding associated with anticoagulants use. Conclusion: The case stresses the importance of maintaining a high degree of suspicion for Behçet's disease in all cases of venous thrombosis. [ABSTRACT FROM AUTHOR]

Blood culture-negative endocarditis is often severe, and difficult to diagnose. The rate of non-documented infective endocarditis has decreased with the advent of molecular biology – improved performance for the diagnosis of bacterial endocarditis with blood cultures sterilized by previous antibacterial treatment – and cardiac surgery – access to the main infected focus, the endocardium, for half of the patients. Blood culture-negative endocarditis are classified in 3 main categories: (i) bacterial endocarditis with blood cultures sterilized by previous antibacterial treatment (usually due to usual endocarditis-causing bacteria, i.e. streptococci, more rarely staphylococci, or enterococci); (ii) endocarditis related to fastidious microorganisms (e.g. HACEK bacteria; defective streptococci – Gemella, Granulicatella , and Abiotrophia sp. – Propionibacterium acnes, Candida sp.): in these cases, prolonged incubation will allow identifying the causative pathogen in a few days; (iii) and the “true” blood culture-negative endocarditis, due to intra-cellular bacteria that cannot be routinely cultured in blood with currently available techniques: in France, these are most frequently Bartonella sp., Coxiella burnetti (both easily diagnosed by ad hoc serological tests), and Tropheryma whipplei (usually diagnosed by PCR on excised cardiac valve tissue). Non-infective endocarditis is rare, mostly limited to marantic endocarditis, and the rare endocarditis related to systemic diseases (lupus, Behçet). [ABSTRACT FROM AUTHOR]

Adamantiades-Behçet's disease (ABD) is a chronic-relapsing multisystemic inflammatory disease with unknown etiology first described by a Greek ophthalmology Benediktos Adamantiades and a Turkish dermatology Hulusi Behçet. Any organ or apparatus may be involved, though more often there is an involvement of oral and genital mucosae as well as ocular lesions, skin features, and vascular findings. Since there is neither laboratory nor radiological pathognomonic test, the diagnosis is basically clinical according to peculiar signs and symptoms of the disease. With the purpose of giving objectivity and homogeneity to the diagnosis, many authors in time introduced a long series of diagnostic and classification criteria for Adamantiades-Behçet's disease. This mini-review provides an overview of published diagnostic/classification criteria., Competing Interests: The author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The handling editor declared a past co-authorship with the author., (Copyright © 2022 Bergamo.)

Objective: The number of citations is used for the scholarly impact of a published article, but it does not always correlate with higher-quality research. Behçet's disease (BD) is a debilitating-blinding disorder with a significant volume of published articles in literature. Our aim was to investigate the references of the 50 most-cited Behçet articles and to evaluate the relationship between altmetric attention score (AAS) and additional metrics. Methods: The Web of Science (WoS) core collection was used to search for the 50 most-cited Behçet articles. Additional metrics and AAS of the reviewed articles and the journals in which the articles were published, were evaluated. Results: A total of 11.372 published articles on BD, between 1975 and 2022, were found. The citation range of 50 highly-cited articles was between 172 and 1322. The "top 50 list" articles were published between 1988 and 2018, and the average age of the article since publication was 18.86 ± 6.08 years. Rheumatology journals were the most published category with 21 articles. There was a weak-to-moderate correlation between AAS and additional metrics. Conclusions: This is the first analysis regarding the AAS of the 50 most-cited articles on BD, which provides useful information about the social impact and characteristics in the academic community. AAS correlates weakly with citation-based quality indexes, and moderately with immediacy index, which evaluates speed. The publication year should be considered when comparing or evaluating the AAS of articles. AAS could be evaluated in a secondary plan for scientific impact analysis., (#x00A9; The Authors.Romanian Society of Ophthalmology.)

Mulher de 38 anos, que recorre à consulta por úlceras orais e genitais com episódio de uveíte anterior aguda bilateral não granulomatosa com oclusão do vaso da retina nasal inferior, adenopatias axilares e HLA B7 e B51 positivos, medicada com prednisolona e inibidor da calcineurina. Quatro dias antes do internamento e cerca de um ano após o diagnóstico de doença de Behçet (DB) emerge quadro de vertigens, náuseas e vómitos acompanhado de cefaleia holocraniana. À observação, diplopia intermitente na levoversão do olhar com desaparecimento da imagem sombra à esquerda na oclusão do olho esquerdo, nistagmo horizontal na dextroversão, Weber lateralizado à esquerda, parésia do VI par esquerdo, marcha com base alargada com ligeiro desvio direito. Tomografia computorizada crânio-encefálica (TC-CE) sem alterações. Ressonância magnética crânio-encefálica (RM-CE) com lesão ao nível da medula oblonga, com extensão à porção posterior da ponte (figura 1). Realizada terapêutica com pulso de metilprednisolona e ciclofosfamida com reversão das alterações objetivadas. Realizada RM-CE de controlo aos 3 meses com franca melhoria, persistindo discretas áreas de hipersinal (figura 2). Atualmente assintomática, sob terapêutica com azatioprina, colquicina e prednisolona 5mg. A utilização de ciclosporina quando a doença tem envolvimento exclusivamente ocular parece ser vantajosa(1)(2), no entanto, considerando a frequência da neuro-toxicidade associada ao fármaco, o uso do mesmo pode dificultar a diferenciação clínica entre os efeitos adversos do fármaco e envolvimento neurológico associado à DB(3). Quando há documentação de lesão do SNC, o uso de ciclosporina é desaconselhado.(4)

The first peruvian pediatric case of Neuro-Behcet, who had seizures, hemiparesis and a magneic nuclear resonance with a pseudotumoral imagine is here presented (Rev Med Hered 1996; 7: 178-181).

Behcet's disease (BD) is a systemic vasculitis usually seen in young adults, which affects both venous and arterial vessels of the vascular system. Its etiology is still unknown. The patognomic symptoms are recurrent oral aphtae accompanies with at least two of these; positive pathergy test, skin lesions, recurrent genital ulcers and lesions in the eye. Urogenital involvement usually consists of genital ulcers, urethritis, epididymitis and recurrent cystitis. Bladder involvement is extremely rare and only several cases have been reported. The findings suggest that neurogenic bladder due to neuro-Behçet disease is characterized uro-dynamically by overactive detrusor in storage phase and detrusor sphincter dyssynergia in voiding phase 12. We aimed to present several aspects of bladder involvement of Behcet disease in this case report. [ABSTRACT FROM AUTHOR]

Behcet's disease (BD) is a systemic vasculitis usually seen in young adults, which affects both venous and arterial vessels of the vascular system. Its etiology is still unknown. The patognomic symptoms are recurrent oral aphtae accompanies with at least two of these; positive pathergy test, skin lesions, recurrent genital ulcers and lesions in the eye. Urogenital involvement usually consists of genital ulcers, urethritis, epididymitis and recurrent cystitis. Bladder involvement is extremely rare and only several cases have been reported. The findings suggest that neurogenic bladder due to neuro-Behçet disease is characterized uro-dynamically by overactive detrusor in storage phase and detrusor sphincter dyssynergia in voiding phase 12. We aimed to present several aspects of bladder involvement of Behcet disease in this case report. [ABSTRACT FROM AUTHOR]

In this study, the authors aimed to evaluate ocular blood flow changes in Behçet disease (BD) with and without thrombotic disease. Ninety eyes of 90 patients with a diagnosis of BD (30 eyes with active uveitis, 23 eyes with inactive uveitis, 25 eyes without ocular involvement, and 12 eyes without ocular involvement and with a history of thrombosis) and 30 eyes of 30 age- and sex-matched control patients without any systemic disease with a total of 120 eyes were evaluated. In all cases, ophthalmic, central retinal, and ciliary artery flow parameters were measured with colour Doppler ultrasonography (CDU). The ocular blood flow parameters of all vessels in patients with active uveitis were found to be affected. All the flow parameters in the CRAs of the study groups were significantly different from the control group ( p < 0.001). Additionally, in non-ocular BD patients with thrombosis, blood flow parameters were affected more than the parameters in non-ocular BD patients without thrombosis and control patients. In conclusion, major haemodynamic changes were observed using CDU in the ophthalmic vessels of ocular Behçet patients. Also, CDU may detect ocular blood flow alterations before initial ocular clinical manifestations appear in BD patients [ABSTRACT FROM AUTHOR]

Background: Behçet disease (BD) is associated with a prothrombotic state of unknown origin that may lead to life-threatening events. Calibrated Automated Thrombogram (CAT) and Rotational Thromboelastometry (ROTEM) are two global haemostasis assays that may reveal new insights into the physiopathological mechanisms of the disease and its procoagulant condition. Methods: 23 BD patients who had no signs or symptoms of current thrombosis and 33 age- and sex-matched controls were included in the study. We performed ROTEM and CAT tests and assessed erythrocyte count, platelet count, platelet contribution to clot formation and plasma levels of tissue-type plasminogen activator, plasminogen activator inhibitor type 1 (PAI-1), fibrinogen, C-reactive protein (CRP), thrombin-antithrombin III complex (TAT), D-dimer and E-selectin (ES). Results: Both ROTEM and CAT tests showed a hypercoagulable state in the BD patients. Plasma levels of PAI-1, fibrinogen, TAT, CRP and ES were significantly increased in this group compared to controls. The disease activity (DA) was significantly correlated with levels of ES and the maximum clot firmness, and this last one, in turn, correlated with rising levels of ES, PAI-1, CRP and fibrinogen. CAT parameters did not correlate with DA or ES. Conclusions: Both ROTEM and CAT tests reveal that patients with BD have a procoagulant state even in the absence of thrombosis. ROTEM test indicates that increased levels of fibrinogen and PAI-1 may be involved in the prothrombotic state of this pathology, while platelets do not significantly contribute. Moreover, CAT assay demonstrate that plasma from BD patients is able to generate more thrombin than controls in response to the same stimulus and that this effect is independent of the DA and the endothelial impairment suggesting the involvement of another factor in the hypercoagulable state observed in BD patients. This study also shows that endothelium activation/damage may be a contributing factor in both the procoagulant and clinical conditions of BD, as shown by the direct correlation between ES levels, ROTEM parameters and DA. [ABSTRACT FROM AUTHOR]

Introduction: Behçet's disease (BD), first explained in 1937 by the Turkish dermatologist Hulusi Behçet, is a complicated multisystem syndrome described by a triad of oral, genital, and ocular ulcers. The present study aimed to investigate existence of some psychological symptoms in the patients suffering from BD. Method: The present research was a cross-sectional one, which was conducted on a sample of 101 randomly selected patients with BD who had been followed up at Shahid Motahari Clinic, Shiraz, Southwest of Iran, from June 2011 to August 2011. All of the patients completed the Symptom Checklist-90-Revised (SCL-90-R) and some demographic information, such as age and gender. All statistical analyses were performed using the SPSS statistical software (v. 17.0). Results: According to the results, 30.7% of the patients were healthy (GSI<0.7), while others (69.3%) had different levels of symptoms (GSI>0.7). In psychiatric symptoms' subscales, somatization (91.7%), anxiety (78%), and depression (77.78%) were the most prevalent symptoms. On the other hand, phobia had the lowest prevalence (34.1%). Based on our findings, the females' average anxiety score was significantly higher than that of the male subjects (p= 0.013). Conclusion: The prevalence of psychological symptoms was remarkable in the patients with BD. Therefore, physicians should monitor their patients in order to detect the possible psychiatric disorders they might develop during their disease. [ABSTRACT FROM AUTHOR]

Oral Diseases (2012) 19, 37-45 Inflammatory ulcerative diseases of the oral mucosa are wide ranging but include especially aphthous and aphthous-like ulceration, vesiculobullous disorders and erosive lichen planus (LP). While most patients with these conditions respond to conventional topical and/or systemic immunosuppressive agents, treatment-resistant cases remain challenging. In these, the use of biologics such as tumour necrosis factor alpha (TNF-α) inhibitors or rituximab may be of benefit. This article reviews the use of biologics in ulcerative oral conditions, highlighting potential benefits, adverse effects and principles of use and future developments. TNF-α inhibitors such as infliximab can be effective in inducing resolution in oral aphthous and aphthous-like ulcers and may be an appropriate therapy in those patients in which disease is severe and refractory to, or patients are intolerant of, traditional immunomodulatory regimens. There would also seem support and rationale for use of biologics (mainly rituximab) in pemphigus but not in oral LP or other oral ulcerative conditions. [ABSTRACT FROM AUTHOR]

Background: Our study aimed to evaluate the efficacy of Tocilizumab and Abatacept for treating Childhood Chronic non-infectious Uveitis (CCU), resistant to anti-tumor necrosis factor (anti-TNF) treatment., Methods: This is a monocentric retrospective charts review study (January 2010-April 2021) recruiting CCU, refractory to anti-TNF. To be included, children should have active uveitis at the time of Tocilizumab (8 mg/kg, every 4 weeks) or Abatacept (10 mg/kg, every 4 weeks). The main outcome was the achievement of ocular remission on treatment defined as the absence of flares for ≥ 6 months., Results: In this study, 18 patients with CCU (14 F), previously treated with Methotrexate and Adalimumab, were enrolled: 15 had juvenile idiopathic arthritis (JIA) (83.3%), 2 idiopathic (11.1%), and 1 Behçet (5.6%). Furthermore, ten patients received Abatacept and 8 patients received Tocilizumab. The mean duration of treatment on Abatacept was 31.6 months (SD ± 30.8), on Tocilizumab 25.25 months (SD ± 17.8). In total, 13 children (72.2%) achieved remission, with a better remission rate for the Tocilizumab group (8/8) compared to the Abatacept group (5/10) (χ 2 5.53, p = 0.019). No difference was evaluated between the two groups in the proportion of patients who showed flares during the treatment (2/6 Abatacept vs. 1/8 Tocilizumab). A significant difference was evaluated in the proportion of patients who flared after treatment discontinuation: 3/3 Abatacept vs. 0/3 Tocilizumab (χ 2 3.8, p = 0.025)., Conclusion: Even though this is a monocentric retrospective study, in a relatively small group, our study suggests a superior efficacy of Tocilizumab over Abatacept for treating anti-TNF refractory CCU., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The reviewer SG declared past co-authorships with one of the authors MM and GS and the absence of any ongoing collaboration with any of the authors to the handling editor., (Copyright © 2022 Maccora, Abu Rumeileh, Curci, de Libero, Marrani, Mastrolia, Pagnini and Simonini.)

Behçet's disease is a chronic multisystemic disorder, characterized by recurrent mouth and genital ulcers and uveitis. We describe the case of a 31 years- old man, with edema in his right leg due to venous thrombosis, recurrent mouth and genital ulcers, and papules-pustules on his knees. He was treated with colchicine and prednisolone. [ABSTRACT FROM AUTHOR]

Copyright of Der Ophthalmologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: Colchicine (COL) has been used in medicine for a long time. It is well recognized as a valid therapy in acute flares of gouty arthritis, familial Mediterranean fever (FMF), Behçet''s disease, and recurring pericarditis with effusion. It has also been used to treat many inflammatory disorders prone to fibrosis, mostly with disappointing therapeutic results. The pharmacotherapeutic mechanism of action of COL in diverse diseases is not fully understood, thought it is known that the drug accumulates preferentially in neutrophils, and this effect is useful in FMF. COL shows a large interindividual bioavailability. Furthermore, interactions with drugs interfering with CYP3A4 dependent enzymes and P-glycoprotein occur and are clinically important. The dosage of COL must be reduced in patients with relevant hepatic and/or renal dysfunction. However, when appropriately used and contraindications have been excluded, oral COL is a safe treatment. [Copyright &y& Elsevier]

Abstract: Bechet''s disease (BD) is an inflammatory, multi systemic disease with spontaneous remissions and relapses similar to various autoimmune diseases. BD leads to organ damage, including the eyes, skin, joints, etc., which produces various clinical manifestations. The central histopathologic characteristic is systemic vasculitis with perivascular inflammatory infiltrates. The etiopathogenesis is unknown, although immunological abnormalities, possibly induced by susceptible microbiological pathogens, have been postulated. [Copyright &y& Elsevier]

Abstract: Objectives: We have study the various associations between Behçet disease and alleles HLA class I and II in Moroccan population. The distribution of HLA alleles involved in the disease is assessed according to clinical signs and sex. Patients and methods: Patients suffering from Behçet''s disease have been compared to healthy controls matched by age and ethnic origin. The HLA typing has been conducted by using microlymphocytotoxicity for the class I (A and B) and molecular biology (polymerase chain reaction-sequence specific primers) for the class II (DR and DQ). Results: In addition to the allele B51, -A2, -B5102, -B58 and -B72 predispose to the Behçet''s disease and the alleles A23, -A33, -B18, -B41 and -B49 have a negative correlation in our Moroccan patients. The alleles A2 and -B72 are specific to men, -A68 and -B58 to women, whereas -B51 and -B5102 are predisposing to the Behçet''s disease of both genders. The allele B51 is related to the mucous manifestations, -B72 to the vascularitis manifestations and -B58 to the cutaneous manifestations. The haplotypes A2-B51 has a high positive correlation with this Behçet''s disease. Concerning class II, none of the alleles DRB1* is implicated in the pathology even though the allele DQB1*02 ensures the patients’ protection. Conclusions: The allele HLA-B51 shows a high positive correlation with the Behçet''s disease. Other genetic factors seem to be implicated in susceptibility or protection against the disease. [Copyright &y& Elsevier]

Copyright of Ginecología y Obstetricia de México is the property of Federacion Mexicana de Ginecologia y Obstetricia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Vulvar ulcers are often misdiagnosed and mistreated as herpetic ulcers. This article describes two other vulvar ulcerous diseases, Behçet’s disease and Lipschütz ulcus, to show the importance of a careful clinical examination and diagnostic testing. [ABSTRACT FROM AUTHOR]

Abstract: Nowadays, Behcet''s disease in child is better described since increasing number of papers are clearly reported the pathology. The goal of this paper is to report the pediatric aspects, especially the importance of fast aggressive, precocious and initial treatment. We report three pediatric cases with various manifestations. Two children among three are 7 and 8 years old respectively; both have severe ocular affections associated to cutaneomucosal lesions evoking the pathology with favorable evolvement; this was the result of initial aggressive and precocious treatment. The third patient was 15 years old. He was only suffering cutaneomucosal lesions. The Behcet''s disease is characterized by its huge clinical polymorphism. The prognosis is still poor and depends on the ocular affection which remains severe in this age range. The initial aggressive and precocious treatment might limit the sequels which are major, especially in the reported age range. [Copyright &y& Elsevier]

The article presents a letter to the editor in response to the article "Neutrophil and lymphocyte responses to oral Streptococcus in Adamantiades-Behçet's disease," in the Number 43, 2006 issue.

PurposeTo evaluate the long-term efficacy and safety of infliximab as treatment for noninfectious posterior uveitis.MethodsAn open-label clinical trial including seven patients (12 eyes) with posterior uveitis refractory to conventional treatment regimens with corticosteroids and at least one immunosuppressive agent. Three intravenous doses of 5 mg/kg of infliximab were administered at weeks 0, 2, and 6. Infliximab infusion was repeated in patients undergoing a relapse of uveitis after initial remission. Improvement was defined as amelioration of visual acuity or disappearance of retinal exudates and/or haemorrhages, decreased macular oedema and/or vitreous opacities. All patients were followed up for at least 36 months.ResultsSix of the seven patients (five diagnosed with Behçet's disease and one diagnosed with sarcoidosis) showed a significant improvement after the first infliximab dose. Only in one patient diagnosed with chronic idiopathic multifocal choroiditis did the drug have no effect, and this patient was withdrawn from the study. At the end of follow-up, one eye had lost one line of vision and three eyes showed improved vision. All eyes had improved in terms of signs of inflammation. No adverse effects of treatment were observed.ConclusionInfliximab is efficient and safe for the long-term management of refractory posterior uveitis, especially in patients with predominant retinal vasculitis and vitritis.Eye (2005) 19, 841–845. doi:10.1038/sj.eye.6701689; published online 24 September 2004 [ABSTRACT FROM AUTHOR]

Behçet's disease is a chronic, relapsing vasculitis that can affect most organ systems. The prevalence varies geographically, and the disease is more common in countries along the ancient Silk Road, including Italy, Turkey, Israel, Saudi Arabia, Iran, China, Korea and Japan. Behçet's is more common in men than in women, and typically affects young adults. The classic finding in Behçet's patients is the presence of recurrent mucocutaneous ulcers, and oral aphthous ulcerations are usually the initial symptom. Other manifestations include genital ulcers, skin lesions, vascular, neurological, articular, and ocular disease. The disease can affect the anterior and/or posterior segments of the eye, and the main manifestations include iridocyclitis, hypopyon, mild to moderate vitreitis, retinal vasculitis and occlusion, optic disc hyperemia, and macular edema. There is no pathognomonic laboratory test in Behtçet's disease, and the diagnosis is based in systemic and ocular clinical findings. Treatment of ocular Behçet is based in corticosteroids and immunosuppressive agents, to suppress acute inflammation and reduce its recurrence frequency. Ocular lesions may improve with immunosuppressive therapy, but usually are not fully reversible, and generally progress over time. The prognosis of anterior uveitis is usually good, but patients with posterior lesions tend to have some degree of visual loss, even with adequate treatment. [ABSTRACT FROM AUTHOR]

Arterial complications of Behcet’s disease rarely affect the thoracic aorta, and the incidence of aortic arch aneurysm is especially low. We present a patient who developed a rapidly expanding aneurysm of the distal aortic arch after 20 years of treatment of Behcet’s disease. Emergency total arch replacement was performed with a favorable outcome. Graft anastomosis to the normal aorta involving all three layers, wrapping of the anastomotic sites with wide felt strips, as well as strict management of systemic inflammation are essential for prevention of late complications associated with graft anastomosis sites. [ABSTRACT FROM AUTHOR]

Examines the frequency of pulmonary vascular problems in patients with Behcet's disease. Frequency of thrombophlebitis, pulmonary artery aneurysms and venous thrombi; Radiologic abnormalities observed in the patients; Use of immunosuppression to treat vasculitis.

Objectives . – To present vascular involvement in Behc¸et’s disease, their prognosis and treatment.Patients . – Among 140 patients with Behc¸et’s disease seen at Hotel-Dieu hospital of Beirut between 1980 and 2000, 18 were found to have vascular involvement. All these patients fulfilled the international study group criteria for diagnosis of Behc¸et’s disease.Results . – Vascular involvement in Behc¸et’s disease was 13% (18/140) and was more frequent in men (13/77 i.e. 17% vs 5/63 i.e. 8%; P = 0,12). Age at diagnosis of vascular involvement of the disease was more precocious in male than female (32 ± 7 years vs 36 ± 7,5 years; P < 0,01). Several sites of involvement were seen in the same patient. We observed 17 thrombophlebitis, 10 arterial thromboses, and one aneurysm. Thrombophlebitis was more frequent in men (82 vs 18%; P < 0,03), while arterial oclusions were more frequent in women (70 vs 30%; P > 0,05). Thrombosis of vena cava and arterial occlusions were the most serious complications. The combination of corticosteroids to anticoagulants and to immunosuppressive therapy was efficacious in superior vena cava syndrome and extracranial arterial occlusions.Conclusion . – Vascular manifestations of Behc¸et’s disease are frequent in our country especially venous lesions. Aneurysms are less frequent than arterial occlusions. Medical treatment could be sufficient in superior vena cava syndrome and arterial occlusions. [Copyright &y& Elsevier]

A case of acute rupture of an abdominal aortic aneurysm in a patient with Behçet's disease is reported. The patient was successfully treated by implantation of an endovascular stent graft. The preinterventional diagnostic procedures and the postinterventional follow-up are described and the benefit and risk vs open surgery is discussed. [ABSTRACT FROM AUTHOR]

Introduction: Cerebral venous thrombosis (CVT) is the most common manifestation of vasculo-Behçet's disease and may be superficial and/or deep localization. The aim of our study was to evaluate the clinical and radiological features of CVT associated with Behçet's disease in our population and to compare findings with previous studies., Materials and Methods: We report a retrospective study of 24 cases of CVT secondary to Behçet's disease, collected between 1999 and 2019 in the neurology department of the Mohamed V Military Hospital (Rabat), the Avicenne Military Hospital (Marrakech) and the Mohamed VI Hospital (Marrakech). The diagnosis of Behçet's disease was made in all cases according to the 2014 International Study Group Criteria for Behçet diseases. Patients received antithrombotic treatment, combined with corticosteroids, in six cases of superficial CVT and with immunosuppressants in cases of deep CVT., Results: Clinical manifestations were dominated by motor deficit in deep localizations and intracranial hypertension in superficial locations. The diencephalic-mesencephalic syndrome was found in 18 patients, whereas intracranial hypertension (71%) and headache (57%) were the most common presentations of superficial CVT. Unlike previous studies, magnetic resonance angiography and conventional angiography performed in our patients confirmed the predominance of deep venous thrombosis (18 cases), whereas superficial CVT was observed only in six cases., Discussion: In our series, the benefit of conventional angiography was undeniable, by confirming the diagnosis of basal vein of Rosenthal thrombosis in 75% of our patients. Short-term outcome was favorable, but sequelae of CVT were noted in 20 patients (75%). The outcome of patients was commonly mRS 02, however 70% of patients presenting with deep CVT at the beginning had a poor outcome (mRS 03) and we did not record any case of venous thrombosis relapse., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

La maladie de Behçet est une vascularite systémique, évoluant par poussées. Son diagnostic est clinique: les manifestations les plus fréquentes sont l'aphtose buccale et génitale et l'uvéite. On peut également observer des atteintes neurologiques, digestives, vasculaires, articulaires ou cutanées. Nous rapportons une observation de maladie de Behçet révélée par une thrombose veineuse cérébrale. [ABSTRACT FROM AUTHOR]