COVID-19 related acute genital ulcer: a case report

ABSTRACT Acute vulvar ulcer (Lipschütz's ulcer) is a rare lesion with local hyperimmunoreactivity triggered by infection, which is characterized by acute, painful, and necrotic ulcerations. This condition is usually found in non-sexually active adolescents, and it resolves spontaneously. We report a case of a 35-year-old woman who was diagnosed with COVID-19 who did not have severe symptoms, but had high levels of D-dimer for 9 days. The COVID-19 diagnosis was followed by the appearance of an acute, necrotic, extremely painful vulvar ulcer, although symptoms caused by COVID-19 had improved. We emphasize the importance of the differential diagnosis to exclude diseases such as Behçet's syndrome, Sexually Transmitted Infections, as well as the presence of viruses that generally trigger Lipschütz's ulcer, such as Epstein-Barr virus and cytomegalovirus. No treatment is usually necessary, however, in the present report due to the pain experienced by the patient, we successfully used oral prednisone.

Tratamento endovascular na doença de Behçet: uma revisão integrativa / Endovascular treatment in Behçet's disease: an integrative review

Resumo A doença de Behçet (DB) é caracterizada por ser uma doença rara, crônica, recidivante, inflamatória e multissistêmica. Quanto ao diagnóstico, não existe nenhum teste universalmente descrito; logo, ele é feito de maneira clínica, por meio da presença da sua tríade clássica de sinais: úlceras orais, úlceras genitais e uveíte (inflamação do trato uveal do olho). O objetivo deste estudo foi avaliar a importância do tratamento endovascular na doença de Behçet, sendo que a revisão de literatura para obter essa resposta foi realizada a partir de 30 artigos publicados entre 2002 e 2021. A DB acomete tanto o sistema venoso quanto o arterial. A ruptura de aneurismas é a principal causa de morte e necessita de tratamento, podendo ser clínico, cirúrgico aberto ou por via endovascular. A cirurgia endovascular vem ganhando espaço no tratamento cirúrgico nos casos de aneurismas arteriais, inclusive nos relacionados com DB, apesar de a terapia de escolha ainda ser controversa.

Abstract Behçet's Disease (BD) is a rare, chronic, relapsing, inflammatory, and multisystemic disease. There is no universally described test for confirming diagnosis, so it is made clinically, on the basis of its classic triad of signs: oral ulcers, genital ulcers, and uveitis (inflammation of the uveal tract of the eye). The aim of this study is to evaluate the importance of endovascular treatment in Behçet's disease. The literature review conducted to achieve this objective covered 30 articles published between 2002 and 2021. Behçet's Disease affects both the venous and arterial systems. Rupture of aneurysms is the main cause of death and thus requires treatment, which can be clinical, open surgical, or endovascular. Endovascular surgery has been gaining ground for surgical treatment of arterial aneurysms, including those related to BD, although the therapy of choice is still controversial.

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)

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.

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.

Evaluation of factors for predicting risk of uveitis recurrence in Behcets disease patients

This study aimed to investigate the predictive factors for uveitis recurrence (UR) risk in Behcet's disease (BD) patients. BD patients (n=164) with a history of uveitis were recruited, and demographic data, clinical features, and laboratory tests were recorded. Uveitis was defined as anterior uveitis, intermediate uveitis, posterior uveitis, panuveitis referring to the "International Uveitis Study Group recommendations for the evaluation of intraocular inflammatory disease". In total, there were 70 UR patients and 94 non-UR patients. Compared to non-UR patients, UR patients appeared to be older and presented with increased uveitis occurrence rate and times within 3 months, oral ulcers occurrence rate, as well as higher concentrations of triglycerides (TG), total cholesterol (TC), low-density lipoprotein (LDL), and serum amyloid A (SAA). Multivariate logistic model disclosed that uveitis occurrence times within 3 months, oral ulcers, TG, LDL, and SAA independently predicted higher risk of UR. Furthermore, receiver operating characteristic curve analysis showed that the combination of uveitis occurrence times within 3 months, oral ulcers, TG, LDL, and SAA exhibited a high predictive value for UR risk with an area under the curve of 0.983 (95%CI: 0.969−0.998). In conclusion, uveitis occurrence times within 3 months, oral ulcers, TG, LDL, and SAA might be potential predictive factors for UR risk in BD patients, which can help in prevention and management of the disease.

Co-existence of Guillain-Barré syndrome and Behcet syndrome: A case report / 北京大学学报(医学版)

A 40-year-old male patient was referred to our department with complains of recurrent oral ulcer for more than 20 years and vulvar ulcer for more than 10 years. He presented with a 3-month history of right external ophthalmoplegia. More than 10 days ago, the patient received ganglioside infusion. And one week ago, he developed numbness and pain of his lambs, and progressive myasthenia, accompanied by right blepharoptosis and dysuria. On exam, motor strength was graded 0/5 in the lower and the upper extremities. Deep tendon reflexes were diminished in extremities. His admission medical examination: hemoglobin (HGB), white cell and platelet counts were normal. C-reactive protein (CRP) was negative. Erythrocyte sedimentation rate (ESR) 53 mm/h. Antinuclear antibody (ANA), anti-dsDNA antibody, anti-Smith antibody, anti-cardiolipin antibody and human leucocyte antigen B51 were all within normal range. The etiological tests of influenza A pathogen, influenza B pathogen, parainfluenza virus, enterovirus and parvovirus were all negative. He tested positive for serum anti-GM1 IgG. Cerebrospinal fluid had a normal white cell count, an elevated protein content. Gram staining, culture and PCR detection for varicella-zoster virus, cytomegalovirus and herpes simplex virus were all negative. Antibodies associated with autoimmune encephalitis and paraneoplastic syndrome were negative in cerebrospinal fluid. Electromyography and nerve conduction studies showed a severe axonal damage affecting motor nerves. No obvious abnormalities were observed in his magnetic resonance imaging of brain and cavernous sinus. The patient was diagnosed with Behcet syndrome complicated with acute Guillain-Barré syndrome. He received intravenous methylprednisolone, intravenous immunoglobulin (IVIg) therapy, plasma exchange and rituximab treatment. After treatment, the patient's muscle strength of limbs was restored to grade 1, blepharoptosis and pain disappeared. The nervous system involvement of Behcet syndrome is relatively rare, especially combined with Guillain-Barré syndrome, which is easy to cause misdiagnosis. The treatment of Behcet syndrome complicated with acute Guillain-Barré syndrome includes the treatment of primary disease, plasma exchange and IVIg therapy. In addition, supportive treatment is very important for such patients. The focus of treatment is to avoid respiratory insufficiency, prevent deep vein thrombosis, monitor cardiac function and hemodynamics. Pain-relieving, physical exercise and psychological support are often under-recognized. The rehabilitation treatment is very important to improve the prognosis and quality of life of patients. What we need to learn is that when the symptoms and signs of the nervous system are difficult to be explained by neuro-Behcet syndrome alone, we should be alert to the possibility of other nervous system diseases.

Coronary involvement in behçet's disease: what are its risks and prognosis? (rare cases and literature review)

Abstract Objective: In our clinic, we aimed to investigate the effect of preoperative risk factors and postoperative complications on reoperation and mortality in cases with Behçet's disease which presents very rare coronary artery involvement. Methods: Thirteen patients with Behçet's Disease who had undergone coronary artery bypass grafting in our center between 2003 and 2015 were analyzed. We evaluated the clinical and laboratory findings, complications and mortality rates of our patients in light of the literature. Results: The mean age was 38.5 (30-55; 3 women). The mean time from onset of Behçet's disease to coronary artery disease was 4,7 (3-11) years. Fifty-four percent of the patients were asymptomatic. Coronary artery disease of these was exposed while peripheral vascular surgery was planned due to complications of Behçet's disease. Symptomatic patients presented angina pectoris (31%), acute coronary syndrome (8%) and arrhythmia (8%). In coronary pathology of patients, distal type obstruction (31%), aneurysm and pseudoaneurysm (31%), proximal segment thrombus (15%), chronic type stenosis and occlusions (31%) were present. Early mortality (15%) was due to acute myocardial infarction while the late mortality (15%) was due to cerebral and gastrointestinal bleeding. Reoperation was due to bleeding in one case on the 1st postoperative day and due to acute pulmonary embolism in another case in the 3rdpostoperative year. Conclusion: In Behçet's disease, coronary artery bypass grafting is a procedure with high mortality, especially in the acute period. The on-pump surgery technique in these cases can be safely performed for multiple bypasses and in patients above 40 years old.

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)

Relationship between erythrocyte sedimentation rate and choroidal and retinal thickness in Behçet's disease / A relação entre a taxa de sedimentação eritrocitária, espessuras de coroide e retina na doença de Behçet

ABSTRACT Purpose: To compare retinal and choroidal thickness in patients with Behçet's disease with and without ocular involvement as well as to evaluate the correlation between erythrocyte sedimentation rate and choroidal thickness among patients with Behçet's disease. Methods: This was a prospective interventional study investigating erythrocyte sedimentation as well as choroidal and retinal thickness among patients with Behçet's disease. Patients who were diagnosed based on The International Criteria for Behçet's Disease with (Group A) or without (Group B) ocular involvement and a matched control group (Group C) participated in the study. Optical coherence tomography measurements and blood tests were performed on the same day. Retinal and choroidal thickness were measured using spectral-domain optical coherence tomography (Spectralis, Heidelberg Engineering, Heidelberg, Germany), and central macular thickness, central subfoveal choroidal thickness, and retinal nerve fiber layer thickness were measured using optical coherence tomography. Results: Average erythrocyte sedimenta­tion values were 9.89 mm/h in Group A, 16.21 mm/h in Group B, and 3.89 mm/h in Group C; average central subfoveal choroidal thickness values were 350.66, 331.74, and 325.95 mm, respectively. Average central macular thickness and retinal nerve fiber layer thickness values of patients in Groups A, B and C were 226.39 and 225.97mm; 234.11 and 92.00 mm; and 97.58 and 99.84 mm, respectively. No significant difference was seen between Group A and B patients in central subfoveal choroidal thickness, central macular thickness, or retinal nerve fiber layer thickness values. Central macular thickness was statistically significantly thinner in Groups A and B than in Group C (p=0.016). Group A had thinning in the nasal quadrant of the retinal and general retinal nerve fiber layers when compared with those in Group C (p=0.010 and 0.041, respectively). A connection could not be established between the erythrocyte sedimentation, central subfoveal cho­roidal thickness, central macular thickness, and retinal nerve fiber layer thickness in the patients with Behçet's disease. Conclusion: The erythrocyte sedimentation rate is typically used to test for activation of Behçet's disease and assess treatment response. In our study, we could not establish a connection between the erythrocyte sedimentation rate and central subfoveal choroidal thickness, central macular thickness, and retinal nerve fiber layer thickness in patients with systematically active Behçet's disease without ocular involvement.

RESUMO Objetivos: Comparar a espessura da retina e da coroide em pacientes com doença de Behçet, com e sem acometimento ocular e avaliar a correlação entre a taxa de sedimentação de eritrócitos e a espessura da coroide em pacientes com doença de Behçet. Métodos: Estudo prospectivo intervencional que investigou a sedimentação de eritrócitos, espessura de coroide e da retina em pacientes com doença de Behçet. Os pacientes que foram diagnosticados com base nos Critérios Internacionais para a Doença de Behçet com (Grupo A) ou sem (Grupo B) envolvimento ocular e um grupo controle correspondente (Grupo C) participaram do estudo. Medidas de tomografia de coerência óptica e exames de sangue foram realizados no mesmo dia. As espessuras da retina e da coroide foram medidas utilizando tomografia de coerência óptica de domínio espectral (Spectralis, Heidelberg Engineering, Hidelberg, Germany) e a espessura macular central, a espessura coroidal subfoveal central e a espessura da camada de fibra nervosa da retina foram medidas usando tomografia de coerência óptica. Resultados: Os valores médios de sedimentação de eritrócitos foram de 9,89 mm/h no Grupo A, 16,21 mm/h no Grupo B e 3,89 mm/h no Grupo C; os valores médios da espessura da coroide subfoveal central foram 350,66, 331,74 e 325,95 mm respectivamente. Os valores médios da espessura macular central e da espessura da camada de fibra nervosa da retina dos pacientes nos grupos A, B e C foram de 226,39, 225,97, 234,11 mm e 92,00, 97,58, 99,84 mm respectivamente. Não houve diferença significativa entre pacientes do Grupo A e B na espessura da coroide subfoveal central, espessura macular central ou valores da espessura da camada de fibra nervosa da retina. A espessura macular central foi estatisticamente significativamente mais fina nos Grupos A e B do que no Grupo C (p=0,016). O Grupo A apresentou afinamento na porção nasal das camadas retiniana e geral da fibra nervosa da retina quando comparado com o Grupo C (p=0,010, p=0,041, respectivamente). Não foi possível estabelecer uma conexão entre a sedimentação dos eritrócitos, a espessura subfoveal central da coroide, a espessura macular central e espessura da camada de fibras nervosas da retina nos pacientes com doença de Behçet. Conclusão: A taxa de sedimentação de eritrócitos é comumente utilizada para testar a ativação da doença de Behçet e avaliar a resposta ao tratamento. Em nosso estudo, não foi possível estabelecer uma conexão entre a taxa de sedimentação de eritrócitos e a espessura da coroide subfoveal central, espessura macular central e espessura da camada de fibras nervosas da retina em pacientes com doença de Behçet sistematicamente ativa sem envolvimento ocular.

High levels of endothelial progenitor cells and circulating endothelial cells in patients with Behçet's disease and their relationship to disease activity

Abstract: Background: Behçet's disease is a multisystemic vasculitis, associated with vascular endothelial dysfunction. Currently, the prognosis is unpredictable, because there is still no valid laboratory marker indicating the disease activity in Behçet's disease. Endothelial progenitor cells and circulating endothelial cells are newly introduced hematological markers which are presumed to take part in the pathogenesis of vasculitis. Objectives: To evaluate the levels of endothelial progenitor cells and subtypes and circulating endothelial cells in patients with Behçet's disease and to describe their relationship with the disease activity. Methods: A total of 45 patients with Behçet's disease and 28 healthy controls were included in the study. Endothelial progenitor cells (CD34+CD133+KDR+ as early endothelial progenitor cells and CD34+KDR+ as late endothelial progenitor cells), and circulating endothelial cells (CD34+CD133+) were measured by flow cytometry. Results: The mean plasma level of endothelial progenitor cells and circulating endothelial cells, vascular endothelial growth factor, matrix metalloproteinase-9, C-reactive protein, and erythrocyte sedimentation rate were significantly higher in patients with Behçet's disease. All of these parameters except circulating endothelial cells were also found to be higher in patients with active disease than in patients with inactive disease. Early endothelial progenitor cells showed significant correlations with C-reactive protein and circulating endothelial cells. Study Limitations: The cross-sectional nature of the study and patient characteristics such as being under treatment, which can affect endothelial progenitor cells numbers. Conclusion: The increase in endothelial progenitor cells may play an essential role in the repair of endothelial injury in Behçet's disease, especially in the active period of the disease. Thus, endothelial progenitor cells can indicate the disease activity. In addition, endothelial progenitor cells and circulating endothelial cells can be used as endothelial repair and injury markers for Behçet's disease, respectively.

Neurobehcet, a propósito de un caso / Neurobechet, about a case

El neurobehcet (NB) es una manifestación poco frecuente y grave de la enfer-medad de Behcet (EB) con diferentes formas de presentación según el aérea del sistema nervioso que se vea afectada. Se presenta el caso de una paciente de sexo femenino de 38 años de edad con historia de larga data de psicosis de posible cau-sa orgánica. Durante su última hospitalización por deterioro funcional y cognitivo se rescata el antecedente de úlceras orales y genitales y un episodio previo de es-cleritis. Se complementa el estudio con punción lumbar, resonancia y angioreso-nancia nuclear magnética de cerebro y antígeno de histocompatibilidad los cuales sugieren el diagnóstico de NB. Se trata con corticoides e inmunosupresores, pero a pesar de mejoría en el líquido cefalorraquídeo no hay mejoría clínica, que se presume sería consecuencia de lo tardío del diagnóstico y tratamiento.

Neurobehcet (NB) is a rare and serious manifestation of Behcet's disease (EB), with different forms of presentation depending on the area of the nervous system involved. A clinical case of a 38-year-old female patient with a long-standing his-tory of psychosis of possible organic cause is presented. During her last hospital-ization due to functional and cognitive impairment the history of oral and genital ulcers and a previous episode of scleritis is obtained. The study is complemented with a lumbar puncture, magnetic resonance, magnetic nuclear angioresonance of the brain and histocompatibility antigen which suggests the diagnosis of NB. It is treated with corticosteroids and immunosuppressants, but despite improve-ment in cerebrospinal fluid there is no clinical improvement, which is presumed to be a consequence of the late diagnosis and treatment.

Hemoptisis como manifestación inicial de la enfermedad de Behçet: casos clínicos / Hemoptysis as the initial manifestation of Behçet's disease: report of two cases

Arterial involvement in Behçet's disease (BD) is less common than venous lesions. The most commonly affected arteries are: the aorta, lower extremity arteries, mesenteric, femoral, coronary, renal, subclavian and pulmonary arteries. The rupture of pulmonary arteries is the main cause of death of patients with EB and the presence of aneurysms is a bad prognostic factor. We report two patients with arterial involvement in BD. A 14 years old male presenting with hemoptysis lasting three days. A chest computed tomography showed an aneurismal dilatation of the right interlobar artery, bilateral intramural thrombi and alveolar hemorrhage. A right lobar pulmonary resection was performed but hemoptysis recurred. Suspecting a BD, prednisone was started and hemoptysis subsided. A 42 years old male presenting with chest pain and hemoptysis. A chest CT scan showed thrombi in pulmonary veins and anticoagulant therapy was started. Two months later he was admitted again due to a massive hemoptysis. The CT scan showed aneurisms in pulmonary arteries, mural thrombi and pulmonary infarction. With the diagnosis of BD, methylprednisolone, followed by prednisone and cyclophosphamide were used, with a good clinical response.

Deterioro neurológico progresivo como forma de presentación inusual del neurobehçet / Progressive neurological deterioration as unusual form of neuro-behçet presentation

La enfermedad de Behçet puede afectar a varios órganos y tiene dentro de sus características la presencia de úlceras recurrentes genitales, orales y la uveítis. El neurobehçet es una de las manifestaciones clínicas más serias e infrecuente de la enfermedad de Behçet y debe ser considerado en el diagnóstico diferencial con enfermedades inflamatorias, infecciosas o desmielinizante del sistema nervioso central. Este reporte de caso describe a una paciente con manifestaciones neurológicas aguda severas, lo cual es inusual en esta enfermedad(AU)

Behçet's disease can affect several organs and the presence of recurrent genital, oral and uveitis ulcers characterize it. Neuro-Behçet's is one of the most serious and uncommon clinical manifestations of Behçet's disease, so it should be taken into account in the differential diagnosis with inflammatory, infectious or demyelinating diseases of the central nervous system. This case report describes a female patient with acute severe neurological manifestations, unusual in this disease(AU)

Orbital myosits in a patient with Behçet's disease / Miosite orbitária em paciente com doença de Behçet

Abstract The etiological diagnosis of orbital myositis (OM) is a challenge. Although it has been associated with previous infectious disease, diseases with autoimmune background, thyroidopathies or with paraneoplasic syndrome, most of them still remain as idiopathic. We describe here a case of OM uncovering a Behçet disease that is considered a rare cause for this kind of eye involvement.

Resumo Trata-se de um caso de ceratite bilateral e simultânea por Acremonium relacionada ao uso intermitente e sem respeitar a lateralidade O diagnóstico etiológico da miosite orbitária (MO) é desafiador. Embora ela tenha sido relacionada com doenças infecciosas prévias, doenças associadas à autoimunidade, tireoidopatias ou síndromes para neoplásicas, a maioria delas ainda permanece como idiopática. Descreve-se aqui, um caso de MO revelando o diagnóstico de doença de Behçet, doença considerada como uma causa pouco comum para este tipo de envolvimento ocular.

Doença de Behçet ocular: a nossa realidade / Behçet disease: our experience

Resumo Objetivo: A doença de Behçet é uma vasculite inflamatória sistémica, de etiologia desconhecida. Pode atingir virtualmente todos os sistemas, sendo as manifestações oculares comuns. O objectivo deste trabalho foi analisar doentes com doença de Behçet ocular quanto aos parâmetros demográficos, manifestações clinicas, terapêutica e principais complicações. Métodos: Estudo descritivo e retrospetivo, que incluiu 11 doentes com o diagnóstico de doença de Behçet ocular, segundo os critérios do ISG, observados na consulta de Inflamação Ocular do Hospital Prof. Doutor Fernando da Fonseca nos últimos 3 anos. Resultados: Identificaram-se 11 doentes, 5 homens e 6 mulheres, todos caucasianos. A idade média ao diagnóstico foi de 33,45±6,49 anos. A manifestação ocular foi o primeiro sinal da doença em 2 doentes. Em 72,7% dos casos as manifestações oculares foram bilaterais. Identificaram-se 4 casos de panuveíte, 3 de uveíte posterior, 2 de uveíte anterior, 1 de queratite e 1 caso de episclerite. O glaucoma e a catarata foram a complicação ocular mais frequente. O tratamento sistémico incluiu a corticoterapia oral em associação com terapêutica adjuvante imunossupressora, sendo os mais utilizados a azatioprina e a ciclosporina. Em 3 doentes houve necessidade de terapêutica biológica com infliximab para controlo da doença. Conclusão: A manifestação ocular mais frequente foi a panuveíte. Esta doença pode condicionar complicações oculares com diminuição irreversível da acuidade visual. A orientação destes doentes exige uma abordagem global e interdisciplinar.

Abstract Objective: Behçet's disease is a systemic inflammatory vasculitis of unknown etiology. It can virtually reach all systems, being common ocular manifestations. The aim of this study was to analyze patients with ocular Behcet's disease regarding demographic parameters, clinical manifestations, therapeutic approach and main complications. Methods: Descriptive and retrospective study, including 11 patients diagnosed with ocular Behcet's disease, according to the ISG criteria, observed in the Ocular Inflammation appointment of the Hospital Prof. Doutor Fernando da Fonseca in the last 3 years. Results: We identified 11 patients, 5 males and 6 females, all caucasian. The mean age at diagnosis was 33.45 ± 6.49 years. Ocular manifestation was the first sign of the disease in 2 patients. In 72.7% of the cases, ocular manifestations were bilateral. There were four cases of panuveitis, 3 of posterior uveitis, 2 of anterior uveitis, 1 of keratitis and 1 case of episcleritis. Glaucoma and cataract were the most frequent ocular complications. Systemic treatment included oral corticosteroid therapy in combination with adjuvant immunosuppressive therapy, and the most commonly used was azathioprine and cyclosporine. In 3 patients there was a need for biological treatment with infliximab to control the disease. Conclusion: The most frequent ocular manifestation was panuveitis. This disease can promote eye complications with an irreversible decrease in visual acuity. Orientation of these patients requires a global and interdisciplinary approach.

Tratamento endovascular de aneurisma de aorta abdominal com erosão de vértebra lombar associada à doença de Behçet: relato de caso / Endovascular repair of abdominal aortic aneurysm with lumbar vertebral erosion in Behçet's disease: case report

A doença de Behçet é uma doença sistêmica, multifatorial e autoimune com diversas manifestações clínicas, entre elas o acometimento vascular. Aneurisma de aorta associado a erosão de vértebra lombar é condição rara na literatura, existindo apenas quatro relatos de caso nas bases de dados da PubMed. O presente artigo relata o caso de paciente do sexo feminino com diagnóstico de Doença de Behçet de longa data e aneurisma sacular de aorta abdominal infrarrenal com erosão de vértebra lombar. O caso foi tratado por meio de técnica endovascular com colocação de endoprótese monoilíaca e enxerto fêmoro-femoral cruzado, devido a limitações anatômicas da bifurcação aórtica. O artigo aborda a raridade desse tipo de apresentação da doença e o desfecho do tratamento e apresenta revisão da literatura sobre esse tema

Behçet's disease is an autoimmune, multifactorial, systemic condition with several clinical manifestations, including vascular disorders. An aortic aneurysm with vertebral erosion is rare in association with this pathology and there are only four case reports listed on the PubMed database. This article reports the case of a female patient with a long-standing diagnosis of Behçet's Disease who developed a saccular infrarenal abdominal aortic aneurysm with lumbar vertebral erosion. Her surgical treatment consisted of endovascular repair with a monoiliac endoprosthesis and a femorofemoral crossover bypass, because of limitations imposed by the anatomy of the aortic bifurcation. This paper discusses the rarity of this presentation of the disease and treatment outcomes and offers a brief review of the relevant literature

Enfermedad de Behçet y embarazo: a propósito de un caso / Behçet's disease and pregnancy: a propos of a case

Introducción: La enfermedad de Behçet es una enfermedad crónica, multisistémica, de causa desconocida, caracterizada clínicamente por la presencia de aftas orales, genitales, manifestaciones sistémicas y prueba de patergia positiva. Es una enfermedad poco frecuente y predomina en el sexo masculino, por lo que su relación con el embarazo es escasamente reportada en la literatura. Objetivo: Mostrar las manifestaciones clínicas, evolución y complicaciones presentes durante el embarazo en una paciente con enfermedad de Behçet. Presentación del Caso: Se presenta el caso de una paciente de 26 años de edad con diagnóstico de Enfermedad de Behçet, la cual presentó complicaciones durante su embarazo que tuvieron como resultado un producto final de la gestación bajo peso. Conclusiones: La Enfermedad de Behçet puede provocar complicaciones sobre el embarazo como las presentadas en este caso, a su vez el embarazo puede producir actividad de la enfermedad, por lo que lograr un adecuado control de esta previo al embarazo y llegar a tener una correcta planificación familiar son vitales para llegar a un feliz término del embarazo en estas pacientes(AU)

Introduction: Behçet's disease is a chronic and multisystem illness, of unknown cause, clinically characterized by the presence of oral sores, genital, systemic manifestations and positive pathergy test. It is a not very frequent illness and prevails in the masculine sex; due to this its relationship with the pregnancy is barely reported in the literature. Objective: To show the clinical manifestations, evolution and complications that are present during the pregnancy in a patient with Behçets illness. Cases presentation: A case of a 26-year-old patient with diagnose of Behçes illness is presented, which show complications during its pregnancy that result at the end of her pregnancy; and underweight. Conclusions: The Behçets illness could cause complications during pregnancy like those presented in this case, in turn the pregnancy can produce activity of the illness, and for that reason, to achieve an appropriate control of the illness before the pregnancy with the aim of having a correct family planning, which is vital to arrive to a happy end of the pregnancy in these patients(AU)