Oral Lesions as the Primary Manifestations of Behçet's Disease: The Importance of Interdisciplinary Diagnostics-A Case Report.

BACKGROUND: Behçet's disease (BD) is a rare chronic auto-inflammatory systemic disease with non-specific oral manifestations, categorised as generalised variable vessel vasculitis that requires an interdisciplinary approach to diagnose due to its phenotypic heterogeneity. Whilst the oral lesions that reoccur in BD underpin the complex diagnostic process, the crucial role of dental professionals is highlighted in a case report summarised herein. We present a case of a 47-year-old male referred to the Oral Medicine Department by a rheumatologist after previous hospitalization for thrombosis of the iliac vein and inferior vena cava. He had elevated inflammatory C-reactive protein biomarker and an increased erythrocyte sedimentation rate. Recurrent episodes of folliculitis, oral and genital ulcers were reported. Clinical examination revealed multiple ulcerations in the oral mucosa. The complementary, histopathological analysis performed to rule out other disorders, based on excisional biopsy, showed non-keratinised stratified squamous epithelium with areas of exocytosis and ulceration. The connective tissue presented an intense mixed inflammatory infiltrate, congested blood vessels, haemorrhage, vasculitis, and HLA-B genotyping identified the expression of HLA-B15, further supporting the BD diagnosis. Treatment was initiated with colchicine, prednisolone, and weekly subcutaneous administration of methotrexate and resulted in the complete remission of oral lesions and no recurrence of other manifestations. CONCLUSIONS: This BD case report emphasizes the importance of a multidisciplinary approach in diagnosing BD, including the use of histopathological assessment and genetic profiling. It highlights the significance of thorough intraoral assessment and referral to a multidisciplinary team for diagnosis. The oral manifestations of BD as the primary symptoms often indicate underlying major systemic pathologies. The authors stress the need for a structured diagnostic algorithm to facilitate timely and effective management of BD.

Abordaje de la enfermedad de Behçet vulvar: comunicación de caso

La enfermedad de Behçet (EB) es un trastorno inflamatorio, multisistémico, recidivante y remitente de etiología desconocida. Una característica clave de la enfermedad es la ulceración orogenital que provoca una morbilidad considerable con gran impacto en la calidad de vida de los pacientes. Su manejo médico consiste en un esquema con colchicina, esteroides o inmunomoduladores. Comunicamos el caso de una paciente con lesiones genitales quien consultó en múltiples ocasiones, recibiendo manejo antibiótico e incluso quirúrgico, con respuesta refractaria a estas intervenciones. Posteriormente, por las características de las lesiones y el comportamiento clínico se sospechó enfermedad de Behçet y se instauró manejo médico con esteroides orales y curaciones con fitoestimulina. Hubo resolución de las úlceras genitales y posteriormente la paciente fue llevada a cirugía para corrección de las sinequias vulvares.

Behçet's disease (BD) is an inflammatory, multisystemic, relapsing-remitting disorder of unknown etiology. A key feature of the disease is orogenital ulceration that causes considerable morbidity with great impact on patient's quality of life. Its medical management consists of a scheme with colchicine, steroids or immunomodulators. We report the case of a patient with genital lesions who consulted on multiple occasions, receiving antibiotics and even surgical management, with refractory response to these interventions. Subsequently, due to the characteristics of the lesion and clinical behavior, Behçet's disease was suspected and medical management with oral steroids and cures with phytostimulin was initiated. The genital ulcers were resolved, and the patient was subsequently taken to surgery for correction of the vulvar synechiae.

Lower Digestive Tract Bleeding in a Patient with Behçet's Disease: A Case Report

Behçet's disease is a chronic, multisystemic, and relapsing inflammatory pathology that frequently manifests with oral and genital ulcers and ocular and skin lesions. It rarely exhibits gastrointestinal involvement, which varies depending on the affected gastrointestinal segment; these have in common the predominance of ulcerated lesions and, consequently, a greater risk of bleeding from the digestive tract. A clinical case of a 28-year-old female patient who consulted for a clinical picture of melenic stools and oral ulcers is described. As a crucial clinical history, she had been diagnosed with Behçet's disease since adolescence, associated with severe gastrointestinal complications. An esophagogastroduodenoscopy was performed with findings of antral erythematous gastropathy and a colonoscopy with a report of ulcerated ileitis. Treatment with azathioprine and corticosteroids was indicated, significantly improving the clinical picture.

La enfermedad de Behçet es una patología inflamatoria crónica, multisistémica y recidivante que se manifiesta frecuentemente con úlceras orales y genitales, y lesiones oculares y cutáneas. En raras ocasiones presenta compromiso gastrointestinal y este varía dependiendo del segmento gastrointestinal afectado; estas enfermedades tienen en común el predominio de lesiones ulceradas y, consecuentemente, un mayor riesgo de sangrado de las vías digestivas. Se describe un caso clínico de una paciente femenina de 28 años que consultó por un cuadro clínico de deposiciones melénicas y úlceras orales. Como antecedente clínico de importancia presentó diagnóstico de enfermedad de Behçet desde la adolescencia asociado a complicaciones gastrointestinales graves. Se realizó una esofagogastroduodenoscopia con hallazgos de gastropatía eritematosa antral y una colonoscopia con reporte de ileítis ulcerada. Se indicó el tratamiento con azatioprina y corticoides con una mejoría significativa del cuadro clínico.

Giant Left Anterior Descending Coronary Artery Aneurysm in a Patient with Behçet's Disease.

Coronary artery involvement is quite rare in the course of Behçet's disease. Complications secondary to coronary artery aneurysms, including rupture, dissection, and myocardial ischemia, may be fatal. In young patients without cardiovascular risk factors, systemic inflammatory vasculitis syndromes should be investigated in case of acute coronary syndrome secondary to dilated coronary arteries. In this report, we present our management strategy in a 31-year-old male patient with Bechet's disease.

Giant Left Anterior Descending Coronary Artery Aneurysm in a Patient with Behçet's Disease

ABSTRACT Coronary artery involvement is quite rare in the course of Behçet's disease. Complications secondary to coronary artery aneurysms, including rupture, dissection, and myocardial ischemia, may be fatal. In young patients without cardiovascular risk factors, systemic inflammatory vasculitis syndromes should be investigated in case of acute coronary syndrome secondary to dilated coronary arteries. In this report, we present our management strategy in a 31-year-old male patient with Bechet's disease.

Distribution of monocytes subpopulations in the peripheral blood from patients with Behçet's disease - Impact of disease status and colchicine use.

The innate immune response has a predominant role in Behçet's disease (BD) pathogenesis, but few studies have assessed monocytes in BD. This study aims to evaluate the profile of monocytes subsets in the peripheral blood of BD patients and healthy controls (HC). Monocytes subsets were identified as classical (CD14+CD16-), intermediate (CD14+CD16dim), and non-classical (CD14dimCD16high) subsets. Patients with BD presented a lower number of total monocytes (p = 0.020) and a lower number (p < 0.0001) of circulating classical monocytes than HC. In contrast, the number of intermediate monocytes was higher in BD patients than HC (p < 0.0001). In BD patients, no associations were observed with the severity of clinical manifestations or therapy. Colchicine was associated with a higher number of non-classical monocytes (p = 0.035). In conclusion, BD patients present an altered distribution of monocytes subsets with a reduction of classical and an increase of intermediate subsets.

Case for diagnosis. Ulcerated lesions: a diagnostic challenge in Behçet's syndrome

Abstract This study reports the clinical case of a 42-year-old patient with ulcerated lesions who was followed up by general practitioners with the diagnosis of recurrent cellulitis. However, when referred to the Dermatology division a diagnosis of Behçet's syndrome was established based on clinical criteria. Although there are defined clinical criteria for this syndrome, sometimes its diagnosis can be challenging, due to lack of knowledge of the disease and extremely heterogeneous clinical phenotype. The authors highlight the potential difficulties in establishing the diagnosis considering the multiple clinical findings during the investigation process, contributing to the risk of increased morbidity and mortality.

Endovascular treatment of aortic saccular aneurysms associated with Adamantiades-Behçet disease.

Adamantiades-Behçet disease is a multisystemic disorder that classically presents with oral and genital ulcers and ocular involvement, with vascular involvement in up to 38% of cases. Aortic involvement is one of the most serious manifestations and is associated with high mortality rates, occurring in 1.5 to 2.7% of cases. We report a case of a saccular abdominal aorta aneurysm in a 49-year-old male patient with complicated Adamantiades-Behçet disease that was treated with endovascular repair.

Vascular involvement in Behçet's disease: the immunopathological process.

Behçet's disease is a rare form of systemic vasculitis that affects small to large vessels. It is characterized by mucocutaneous, pulmonary, cardiovascular, gastrointestinal, and neurological manifestations. Its clinical presentation is quite wide, ranging from milder cases to severe cases, with multisystemic involvement, characteristically with exacerbations and remissions. Its etiopathogenesis is still unclear, although there is evidence of genetic, environmental, and immunological factors, such as the association with the HLA-B51 allele. In conjunction, all of these point to an abnormal immunopathological process, with activation of cells of innate and adaptive immunity, such as NK cells, neutrophils, and T cells, which generate specific response patterns and cytokines capable of generating mediators that can damage and inflame blood vessels, resulting in venous and arterial occlusions and/or aneurysm formation.

Optical coherence tomography angiography findings in patients with ocular and non-ocular Behcet disease / Achados de angiografia por tomografia de coerência óptica em pacientes com doença de Behçet com e sem acometimento ocular

ABSTRACT Purposes: To evaluate the optical coherence tomography angiography findings in patients with Behçet disease with and without ocular involvement. Methods: A total of 40 patients with Behçet disease and 30 healthy controls were enrolled in the study. Retinal vessel density in the superficial capillary plexus and deep capillary plexus, foveal avascular zone area and perimeter, acirculatory index, foveal density, and nonflow area in the superficial retina were automatically measured using the optical coherence tomography angiography software AngioVue and compared between the groups. Results: The mean parafoveal and perifoveal vessel densities in the superficial capillary plexus and deep capillary plexus and foveal density were significantly lower in the eyes with Behçet uveitis compared to the eyes without Behçet uveitis and eyes of the healthy controls. In the eyes with Behçet uveitis, logMAR visual acuity showed a moderate correlation with parafoveal and perifoveal vessel densities and foveal density (r=-0.43, p=0.006; r=-0.62, p<0.001; r=-0.42, p=0.008; respectively). Conclusion: Behçet disease with posterior uveitis was associated with significant perifoveal and parafoveal vascular decrements in the superficial and deep retina.(AU)

RESUMO Objetivo: Avaliar achados de angiografia por tomografia de coerência óptica em pacientes com doença de Behçet com e sem acometimento ocular. Métodos: Foram incluídos 40 pacientes com doença de Behçet e 30 controles saudáveis. A densidade vascular retiniana nos plexos capilares superficial e profundo, a zona avascular foveal, o índice de circularidade, a densidade foveal e a área sem fluxo da retina superficial foram medidos automaticamente, através do software AngioVue para angiografia por tomografia de coerência óptica, e comparados entre os grupos. Resultados: A densidade vascular parafoveal e perifoveal média nos plexos capilares superficial e profundo, bem como a densidade foveal, foram significativamente menores nos olhos com uveíte de Behçet em comparação com os olhos sem uveíte de Behçet e os olhos dos controles saudáveis. Nos olhos com uveíte de Behçet, a acuidade visual logMAR mostrou correlação moderada com a densidade vascular parafoveal e perifoveal e com a densidade foveal (respectivamente, r=-0,43, p=0,006; r=-0,62, p<0,001; e r=-0,42, p = 0,008). Conclusão: A doença de Behçet com uveíte posterior foi associada a decréscimos significativos da vascularização perifoveal e parafoveal na retina superficial e profunda.(AU)

Case for diagnosis. Ulcerated lesions: a diagnostic challenge in Behçet's syndrome.

This study reports the clinical case of a 42-year-old patient with ulcerated lesions who was followed up by general practitioners with the diagnosis of recurrent cellulitis. However, when referred to the Dermatology division a diagnosis of Behçet's syndrome was established based on clinical criteria. Although there are defined clinical criteria for this syndrome, sometimes its diagnosis can be challenging, due to lack of knowledge of the disease and extremely heterogeneous clinical phenotype. The authors highlight the potential difficulties in establishing the diagnosis considering the multiple clinical findings during the investigation process, contributing to the risk of increased morbidity and mortality.

Síndrome de Behçet ou úlcera de Lipschütz: desafio diagnóstico / Behçet's syndrome or Lipschütz ulcer: diagnostic challenge

A ocorrência de úlceras genitais em adolescentes e mulheres jovens tem impacto emocional para as pacientes e seus familiares, pela frequente associação com uma possível etiologia de transmissão sexual. Porém, úlcera de Lipschütz e síndrome de Behçet não têm etiologia infecciosa e devem ser lembradas como possíveis diagnósticos diferenciais. O diagnóstico dessas duas patologias é clínico e pode ser desafiador. Dessa forma, foi realizada uma revisão na literatura com o objetivo de comparar as duas entidades. A úlcera de Lipschütz é causada por uma vasculite local e caracteriza-se pelo surgimento súbito de úlceras na vulva ou vagina inferior. Já a doença de Behçet é causada por vasculite sistêmica, com episódios de remissão e exacerbação, que pode envolver quase todos os sistemas orgânicos. Em ambos os casos, é essencial o referenciamento para reumatologia. O tratamento objetiva suprimir exacerbações, controlar a dor e prevenir infecção secundária.(AU)

The occurrence of genital ulcers in adolescents and young women have an emotional impact for the patient and their families, due to the frequent association of its etiology with a sexually transmitted disease. However, Lipschütz ulcer and Behçet's syndrome do not have an infectious etiology and should be remembered as a possible differential diagnoses. As the diagnosis of these two pathologies is clinical and can be challenging, a review of literature was carried out. The objective of this review of literature was to compare both diseases. Lipschütz ulcer is caused by local vasculitis and is characterized by the sudden appearance of ulcers in the vulva or lower vagina. Behçet's syndrome is caused by systemic vasculitis, with episodes of remission and exacerbation, which can affect almost all organ systems. In both cases, referral to rheumatology is essential. Treatment aims to suppress exacerbations, control pain and prevent secondary infection.(AU)

Acometimento vascular na doença de Behçet: o processo imunopatológico / Vascular involvement in Behçet's disease: the immunopathological process

Resumo A doença de Behçet constitui uma forma rara de vasculite sistêmica, que acomete de pequenos a grandes vasos. É caracterizada por manifestações mucocutâneas, pulmonares, cardiovasculares, gastrointestinais e neurológicas. Sua apresentação clínica é bastante ampla, variando de casos mais brandos a casos graves, com acometimento multissistêmico, caracteristicamente com exacerbações e remissões. Suas causas ainda são desconhecidas; entretanto, há evidências genéticas, ambientais e imunológicas, como a associação com o alelo HLA-B51. Todas essas, em conjunto, apontam para um processo imunopatológico anormal, com ativação de células da imunidade inata e adaptativa, como as células natural killer, neutrófilos e células T, que geram padrões de respostas e citocinas específicos capazes de gerar mediadores que podem lesionar e inflamar o sistema vascular, resultando em oclusões venosas, arteriais e/ou formação de aneurismas.

Abstract Behçet's disease is a rare form of systemic vasculitis that affects small to large vessels. It is characterized by mucocutaneous, pulmonary, cardiovascular, gastrointestinal, and neurological manifestations. Its clinical presentation is quite wide, ranging from milder cases to severe cases, with multisystemic involvement, characteristically with exacerbations and remissions. Its etiopathogenesis is still unclear, although there is evidence of genetic, environmental, and immunological factors, such as the association with the HLA-B51 allele. In conjunction, all of these point to an abnormal immunopathological process, with activation of cells of innate and adaptive immunity, such as NK cells, neutrophils, and T cells, which generate specific response patterns and cytokines capable of generating mediators that can damage and inflame blood vessels, resulting in venous and arterial occlusions and/or aneurysm formation.

Tratamento endovascular de aneurisma sacular aórtico associado à doença de Adamantiades-Behçet / Endovascular treatment of aortic saccular aneurysms associated with Adamantiades-Behçet disease

Resumo A doença de Adamantiades-Behçet é uma desordem multissistêmica que se apresenta classicamente com úlceras orais e genitais e envolvimento ocular, podendo o acometimento vascular ocorrer em até 38% dos casos. O envolvimento aórtico é uma das manifestações mais severas e está associado a altas taxas de mortalidade, ocorrendo em 1,5 a 2,7% dos casos. Relatamos um caso de aneurisma sacular de aorta abdominal em um paciente de 49 anos com doença de Adamantiades-Behçet complicada, tratada por correção endovascular.

Abstract Adamantiades-Behçet disease is a multisystemic disorder that classically presents with oral and genital ulcers and ocular involvement, with vascular involvement in up to 38% of cases. Aortic involvement is one of the most serious manifestations and is associated with high mortality rates, occurring in 1.5 to 2.7% of cases. We report a case of a saccular abdominal aorta aneurysm in a 49-year-old male patient with complicated Adamantiades-Behçet disease that was treated with endovascular repair.

Enfermedad de Behcet: un reto diagnóstico en reumatología. Descripción de una serie de casos y revisión de la literatura / Behcet's disease: a diagnostic challenge in rheumatology. Description of a case series and a review of the literature

RESUMEN La enfermedad de Behcet es una entidad clínica autoinflamatoria, de etiología desconocida, generalmente con compromiso sistémico, con un patrón de exacerbación y remisión frecuente que se asocia a retraso en el diagnóstico. El diagnóstico de esta enfermedad es complejo, por esta razón presentamos 4 casos de pacientes con enfermedad de Behcet, que durante el abordaje clínico fueron consideradas otras enfermedades de naturaleza autoinmune. La revisión integrada de la historia clínica, la aparición de úlceras orales y genitales, así como el estudio de tipificación del complejo mayor de histocompatibilidad (HLA) permitieron diagnosticar la enfermedad de Behcet.

ABSTRACT Behcet disease is a rare autoinflammatory disorder of unknown aetiology and is characterised by systemic manifestations with an exacerbation-remission pattern, often associated with diagnostic delay. The diagnostic approach to this disease is complex. A report is given on four cases of patients fulfilling the diagnostic criteria for Behcet disease. Other autoimmune rheumatic diseases were considered in the clinical approach. A meticulous clinical evaluation, taking into consideration relapsing aphthous ulcers in oral mucosa and genitalia, and HLA typing allowed a proper diagnosis of Behcet disease to be made.

Síndrome de Behçet ­ os desafios do diagnóstico: uma revisão sistemática / Behçet Syndrome ­ the challenges of diagnosis: a systematic review

A Doença de Behçet (DB) é uma afecção entendida por ser inflamatória, multissistêmica,de causa ainda desconhecida, apresentando sintomas como úlceras orais, genitais, uveíte, lesões cutâneas e vasculites recorrentes, sendo mais frequente nos países que se estendem desde o Mediterrâneo até o extremo Oriente. Reconhecida atualmente como uma doença autoimune, a DB parece agregar elementos genéticos do portador e fatores ambientais desencadeantes. Para o estabelecimento de critérios internacionais para um diagnóstico, protocolos clínicos foram desenvolvidos para auxiliar a atividade da doença assim permitindo a padronização das pesquisas na área, ainda que não existam alterações laboratoriais ou histopatológicas definidas da doença. O diagnóstico depende de uma avaliação clínica criteriosa que, quando precoce, é determinante para o prognóstico.Neste artigo são revistos dados clínicos para o diagnóstico da Doença de Behçet.

Behçet's disease (DB) is an affection understood to be multisystemic inflammation of an as yet unknown cause, presenting symptoms such as oral ulcers, genitalia, uveitis, cutaneous lesions and recurrent vasculitis. It is more frequent in countries stretching from the Mediterranean to the Far East. Currently recognized as an autoimmune disease, DB appears to aggregate carrier genetic elements and environmental triggering factors. The establishment of international criteria for a diagnosis, clinical protocols were developed to aid the activity of the disease thus allowing the standardization of research in the area, although there are no laboratory or histopathological changes defined disease, the diagnosis dependent on a careful clinical evaluation that when are determinants for the prognosis. In this article we review clinical data for the diagnosis of Behçet's Disease.

Prevalence and demographic characteristics of Behcet disease in Colombia: data from the national health registry 2012-2016.

To estimate prevalence and describe the main demographic characteristics of Behcet disease in Colombia. Cross-sectional study, based on official Ministry of Health registry data. 523 cases of Behcet disease (ICD-10 code: M352) were reported between 2012 and 2016, for a prevalence in people over 18 years old of 1.10 per 100,000 inhabitants, of which 68% are women, with a female-to-male ratio of 2.2:1, with greater prevalence within the 45-49 age group. This is the first study that shows demographic and epidemiological information on Behcet disease in Colombia. Prevalence seems to be low when compared with other similar studies in the region.

Enfermedad de neuro-Behçet con paquimeningitis en una niña / Neuro-Behçet disease presented with pachymeningitis in a child

La enfermedad de Behçet (EB) es un trastorno de vasculitis sistémica poco frecuente, de etiología desconocida, que se caracteriza por la presencia de aftas bucales, úlceras genitales y uveítis recurrentes. Afecta los sistemas nerviosos central y periférico; raramente se produce durante la niñez. La meningitis aséptica aguda aislada es muy poco frecuente. En este artículo, se describe el caso de una paciente de 14 años con diagnóstico de enfermedad de Behçet con afectación neurológica (neuro-Behçet). La paciente tenía cefalea aguda, diplopia, papiledema e irritación meníngea. Tenía antecedentes de úlceras bucales recurrentes. Las imágenes por resonancia magnética de cerebro revelaron paquimeningitis. Los hallazgos en el líquido cefalorraquídeo fueron pleocitosis y aumento de la presión. Pese al tratamiento médico, sus síntomas no se resolvieron. No se detectó uveítis y la prueba de patergia fue negativa. El alelo HLA-B51 fue positivo. Se consideró que los hallazgos apuntaban a la poco frecuente enfermedad de Behçet con afectación neurológica. La paciente mejoró drásticamente luego del tratamiento con corticoesteroides. En el diagnóstico diferencial de meningitis, se debe considerar la EB, a menos que se demuestre la presencia de un agente infeccioso. Hasta donde sabemos, anteriormente no se había descrito un caso de paquimeningitis con neuro-Behçet en la población pediátrica.

Behçet's disease (BD) is a rare systemic vasculitis disorder of unknown etiology characterized by recurrent oral and genital apthae and uveitis. It involves the central or peripheral nervous system; occurs rarely during childhood. Isolated acute aseptic meningitis is extremely uncommon. We report here a case of Neuro-Behçet disease (NBD) diagnosed in a 14-year-old girl. The patient presented acute headache, diplopia, papilla edema, and meningeal irritation. She had a history of recurrent oral ulcers. Brain magnetic resonance imaging revealed pachymeningitis. Pleocytosis and pressure increase were the cerebrospinal fluid findings. Although medical therapy, her complaints were not resolved. Uveitis was not detected, pathergy test was negative. HLA-B51 allele was positive. The findings were considered to unusual NBD. The patient improved dramatically after steroid therapy. BD should be considered in differential diagnosis of meningitis unless an infectious agent is demonstrated. To our knowledge, a case of pachymeningitis with NBD, was not described in children.

Síndrome de Behçet complicado con trombosis venosas profundas en miembros inferiores / Behçet syndrome complicated with deep vein thrombosis in lower limbs

En este trabajo se presentó a un paciente de 32 años que ingresó por pérdida de peso, decaimiento, orinas turbias y dolor en región inguinal derecha. Se describió la evolución clínica, los estudios imagenológicos y de laboratorio que permitieron diagnosticar la presencia del Síndrome de Behçet. Los principales signos presentados por el paciente fueron: ulceraciones bucales recurrentes, la presencia de uveítis anterior y trombosis venosas profundas en miembros inferiores. El cuadro clínico del paciente y la valoración conjunta de varias especialidades médicas constituyeron los factores claves para establecer el diagnóstico definitivo. La evolución del paciente fue satisfactoria con el tratamiento de prednisona y anticoagulantes(AU)

This paper reports a 32-year-old male patient who was hospitalized for weight loss, decay, cloudy urine and pain in the right inguinal region. The clinical evolution, imaging and laboratory studies allowed diagnosing Behçet Syndrome. The patient showed main signs as recurrent mouth ulcerations, anterior uveitis and deep vein thrombosis in the lower limbs. The clinical condition of this patient and the joint assessment of various medical specialties were the key factors in establishing the definitive diagnosis. The patient´s evolution was satisfactory treated with prednisone and anticoagulants(AU)