Doença de Behçet, anticorpos anticardiolipina, forame oval patente e síndrome de Sweet ? uma associação incomum / Behçet?s disease, anticardiolipin antibodies, patent foramen ovale, and Sweet syndrome ? an uncommon association

Doença de Behçet é condição inflamatória e autoimune infrequente, caracterizada por úlceras orais recorrentes e dois ou mais dos seguintesachados ? úlceras genitais, alterações oculares, lesões cutâneas e teste de patergia positivo. A ocorrência de vasculites e de anticorpos antifosfolípides pode contribuir para o aparecimento de tromboembolismos na doença de Behçet. Mulher de 49 anos, com doença de Behçet associada com diagnóstico prévio de síndrome de anticorpos antifosfolípides, apresentou erupção cutânea consistente com a síndrome de Sweet em um local de punção venosa. Foi introduzida prednisona 20mg/dia ao tratamento com warfarina 5 mg/dia que já utilizava, e a resposta satisfatória foi rápida. Pesquisa de sangue oculto foi positiva nas fezes, com concentração de antígeno carcinoembrionário discretamente elevada, e a colonoscopia revelou pólipos no cólon e no sigmoide. O câncerde cólon é descrito em portadores de doença de Behçet com lesões intestinais. Forame oval patente com shunt esquerdo-direito foi achado incidental no presente caso, e essa anomalia propicia embolias paradoxais em pacientes com estados hipercoaguláveis. A associação de doença de Behçet com síndrome de Sweet é raramente descrita e, embora sem o necessário esclarecimento, alguns autores admitem a possibilidade de mecanismo etiopatogênico comum a essas entidades.

Behçet?s disease is an inflammatory and autoimmune condition, characterized by recurrent oral ulcers and, at least, two of the following findings ? genital ulcers, ocular changes, cutaneous lesions, and positivetest for pathergy. The occurrence of vasculitis and antiphospholipid antibodies may contribute to thromboembolisms in Behçet?s disease. A 49-yearold woman with Behçet?s disease associated with a previous diagnosis of antiphospholipid antibodies syndrome presented with an abrupt cutaneous eruption consistent with the Sweet syndrome at the site of venipuncture. Prednisone 20 mg/day was introduced to the treatment with warfarin 5 mg/day that she was undergoing, resulting in a rapid favorable response. Fecal occult blood test was positive, and the level ofcarcinoembryonic antigen was mildly elevated, and the colonoscopy evaluation revealed colon and sigmoid polyps. Colon cancer may develop in patients with intestinal lesions of Behçet?s disease. Patent foramen ovale with left to right shunt was an incidental finding in the present case study, and this anomaly predisposes to paradoxal embolism in patients with thrombophilic conditions. Association between Behçet?s disease andSweet syndrome has been rarely described and, although without the necessary clarification, some authors believe in a possible common etiopathogenic mechanism.

Doença de Behçet e a visão do otorrinolaringologista / Behçet’s disease as viewed by the otorhinolaryngologist

A doença de Behçet é um distúrbio inflamatório autoimune, que acomete pequenos vasos sanguíneos. O diagnóstico da síndrome é clínico e necessita a presença de úlceras orais recorrentes, úlceras genitais e desordens oculares. No entanto, diversos sistemas podem ser afetados. Na avaliação otorrinolaringológica, as lesões orais são os sintomas mais frequentes da doença. O paciente do caso desenvolveu odinofagia no pósoperatório de cirurgia para correção de aneurisma. Ao exame, foram identificadas úlceras orais e lesão ulcerada na base peniana. Foi aventada hipótese diagnóstica de doença de Behçet e iniciado terapia com corticoide e colchicina. Embora a doença de Behçet não seja frequente na prática diária, os profissionais médicos devem estar atentos a sua apresentação

Behçet’s disease is an inflammatory autoimmune disorder that affects small blood vessels. The diagnosis of the syndrome is clinical and requires the presence of recurrent oral ulcers, genital ulcers, and ocular disorders. However, several systems may be affected. On otorhinolaryngological evaluation oral lesions are the most frequent symptoms of the disease. The patient described here developed postoperative odynophagia after surgery for aneurysm. On examination oral ulcers and ulcerated lesion on the penile base were found. A diagnosis of Behcet’s disease was hypothesized and therapy with corticosteroids and colchicine was initiated. Although Behcet’s disease is rare in everyday practice, medical professionals should be aware of its presentation

Aneurisma volumoso da artéria pulmonar em paciente portador de doença de Behçet: relato de caso / Huge aneurism of the pulmonary artery in patient with Behçet's disease: case report

JUSTIFICATIVA E OBJETIVOS: A doença de Behçet caracteriza-se pela presença de lesões cutâneas, história de ulcerações orais, observação de patergia, envolvimento ocular do tipo uveíte e alterações vasculares. Constitui-se em afecção rara no nosso meio, inscrevendo-se no capítulo das vasculites sistêmicas primárias. RELATO DO CASO: Descrição de manifestação vascular pulmonar incomum, caracterizada pelo desenvolvimento de volumoso aneurisma na circulação arterial pulmonar em paciente com doença de Behçet. CONCLUSÃO: A presença de aneurisma da artéria pulmonar é manifestação rara, porém observada na doença em questão.(AU)

BACKGROUND AND OBJECTIVES: Behçet `s disease is a rare affection characterized by skin lesions, history of aftous ulcers, pathergy, ocular involvement like uveitis, as well as venous and arterial compromise. CASE REPORT: A patient with various systemic manifestations of Behçet’s disease, including a huge aneurysm in the left pulmonary artery. CONCLUSION: The appearance of this unusual vascular alteration is considered a rare manifestation of Behçet’s disease.(AU)

A Case of Multiple Esophageal Ulcerations in Behcet's Disease / 대한소화기내시경학회지

The diagnostic criteria of Behcet' s disease - which requires the presence of recurrent oral ulceration plus any two of recurrent genital ulceration, typical defined eye lesions, typical defined skin lesions or a positive pathergy test - was proposed by the International Study Group for Behcet' s Disease (ISGBD). Intestinal Behcet' s disease most commonly affects the ileocecal region, but esophageal involvement of Behcet' s disease is rare, only 8 cases have previously been reported in Korea. A 23-year old female who had a 10-year history of recurrent oral and genital ulcer and recurrent erythematous skin eruption, was admitted to our hospital because of dysphagia and substernal pain. Endoscopic exami-nation showed multiple small sized ulcers on oral cavity and two diffuse deep ulcers surrounded with irregularly nodular and hyperemic edematous mucosa at distal esophagus. The pathologic finding was subepithelial accumulation of chronic inflammatory cells, especially around the vessels. So she was treated with steroid under the diagnosis of esophageal involvement of Behcet' s disease. She has been followed in improvement status without recurrence.

A Case of Behcet's Colitis in Children / 대한소화기내시경학회지

Behcet's disease consists of a triad of relapsing inflammatory disease of the eye (iridocyclitis) with painful and recurrent oral and genital ulcerations. Arthritis, thrombophlebitis, neurologic abnormalities, fever, and colitis are associated clinical manifestations. The disease affects predominantly young adults and is very rare in children, especially those under 10 years of age. The proportion of Behcet's colitis in Behcet's disease is about 12%. Ulcerations are localized or diffuse, with the majority occuring in the ileocecal region. Extension to the serosal surface may result in perforation. The frequent complaints are abdominal pain, nausea, vomiting, diarrhea, hematochezia, loss of appetite, loss of weight gain, distention and palpable abdominal mass. The disease is complicated by intestinal obstruction, perforation, fistula formation, abscess formation and hemorrhage. We experienced a case of Behcet's colitis in a 9-year-old boy showing symptoms of abdominal pain, tenderness, and diarrhea. Thus, we report a case of Behcet's colitis with brief review of related literatures.

Analysis of Typing in Patients with Uveitis in Korean Population

In order to evaluate association of particular HLA typing with certain uveitis in Korean population, HLA antigens were analyzed in 114 uneitis patients(acute anterior uveitis: 32 cases, Behcet`s disease: 25 cases, intermediate uveitis: 19 cases, Vogt-Koyanagi-Harada (V-K-H) syndrome: 10 cases, retinal vasculitis: 12 cases, Eale`s disease: 3 cases, posterior uveitis: 9 cases, pan.uveitis: 4 cases). The stronger association between acute anterior uveitis and HLA-B27 was statistically significant, and this result was similar to reports in other ethnic groups. Also, the association between V-K-H syndrome and HLA-DR4 showed same results. But the high frequency of HLA-DR7 in the patients with V-K-H syndrome was unque in patients of Korean popjlation and statistically significant. The association between HLA-A2 and posterior uveitis was high in patients of Korean population and statistically significant. Behcet`s disease was stronger association with HLA-B51 but not statistically significant and much weaker association than reports in Japanese group. Although many similarities of associations between particular uveitis and HLA typing were detected as compared with other ethnic groups, distinctive HLA associations were demonstrated in Korean population. Additional cases and long-term follow-up are required to confirm the association with HLA typing and the relationship with prognosis including clinical and laboratory variabilities.

Clinical Evaluation of Behcet`s Disease Report of 85 Cases

To evaluate the clinical characteristics of Behcet`s disease, we reviewed the clinical record of the patient visited Chonnam University Hospital from January, 1987 to December, 1995. Among all 85 patients, the majority of them were in the fourth decade (42.4%) and 49 patients (58.9%) were female. Oral ulcer (81.2%) was the most frequent initial symptom. According to the criteria established by the Behcet`s Disease Research Committee of Japan, incomplete type was the ost common (43.5%) and the most frequent major and minor symptom was oral ulcer (95.3%) and arthritis (21.2%), respectively. Ocular lesions ppeared in 39 (45.9%), and 19 patients (48.7%) had two or more ocular esions. The most common symptom was iritis (70.7%) and male was more commonly affected than female. The mean interval between initial symptom and ocular symptom was 45.8 months. Among 43 eyes of 26 patients with follow-up of 3 months or more, 14 (32.6%) showed an impairment of two or more lines in visual acuity and 21 (48.8%) eyes had inal visual acuity of 0.3 or less. As similar to other reports, this result suggests Behcet`s disease is achronic, inflammatory disorder characterized by poor visual prognosis.

A Study of Natural Killer Cell Activities in Patients with Behcet's Disease / 대한피부과학회지

Recently, many investigators demonstrate that immunologic mechanisms seem to play an important role in the pathogenesis of Behcet's disease. The present study was undertaken to see the possible roles of NK cell activity in the pathogenesis of Behcet's disease and relationship among NK cell and T-cell and T-subsets. Authors evaluated NK cell activities in 14 patients and T cell, t-subsets in 7 patients. The results were as follows ; 1. The mean value of NK cell activities showed no statistically significant difference between patients group(57.1+/-15.8) and normal healthy control group(56.1+/-16.2). 2. The mean value of T cell(T), T-subsets(T,T)in 7 patients group 74.4+/-9.8, 41. 6+/-8.0, 24.4+/-10.1 showed no statistically significance compared with the mean value of normal healthy control group 67.5+/-4.3, 39.7+/-5.6,26.3+/-6.0 respectively. 3. The mean value of NK cell activities showed no statistically significant difference between 7 complete type patients' group(49.6+13.1) and 7 incomplete type patients' group(64.4+15.6)