Intestinal Behcet’s : A Rare Presentation of Behcet’s Disease

We herein present a case of 48 years old female patient presented with fever, bloody diarrhea followed by palpable purpuric rash over the body along with recurrent oral and genital ulceration. These were associated with history of symmetric polyarthralgia. On examination moderate anemia, signs of anterior uveitis were found. In blood parameters thrombocytopenia along with elevated Erythrocyte Sedimentation Rate, C-Reactive Protein were noted. On further investigations the serological tests were found to be negative for Dengue, Chikungunya, HIV, HBV, HCV. Complement C3 found to be low. Colonoscopic biopsy is diagnostic of Indeterminate Crohn’s Disease with IgA, G, M, C3, Fibrinogen immunostaining in skin biopsy. ANA, P-ANCA, C-ANCA were found to be nonreactive for the patient. All of the above mentioned points were pointing towards Behcet’s disease. For confirmation, Anti Saccharomyces Cerevisae Antibody was found to be positive . Skin pathergy test was positive. So, we diagnosed this case as behcet’s disease.

Chronic arthritis in Juvenile Behcet’s Syndrome: a rare case

Behçet’s syndrome is a chronic multisystem vasculitis characterized by mucocutaneous, articular, neurological, gastrointestinal and ophthalmological lesions. Behcet’s syndrome is a disease of uncertain etiology. It may be due to immune dysregulation including circulating immune complex, autoimmune cytokines and heat shock proteins are major factor in the pathogenesis of Behcet’s syndrome. Patient’s positive pathergy test suggests a diagnosis of Behcet’s disease. Corticosteroids and immunosupressive drugs are the first line of treatment. This is a case report of juvenile Behcet’s syndrome in a thirteen year old girl associated with arthritis.

Report of Bechet's disease

Behcet's disease is a chronic, multisystemic disorder which is more frequently seen in the Mediterranean basin, Middle East, and Far East. The mean age at the first onset is third decades. In large series of patients, men predominate over women. Infectious agents, immune mechanisms, and genetic factors are implicated in the etiopathogenesis of the disease. Eyes, skin, joints, the oral cavity, blood vessels, and central nervous system are usually involved, although less frequently the heart, lung, kidney may be affected. The prognosis of the disease has been improved because of early diagnosis and suitable treatment. Local remedies and systemic administration of colchicine, corticosteoids, immunosuppressives, and other agents have been applied. Pathergy, or skin hypersensitivity to needle puncture has been reported as a diagnostic test for Behcet's disease. We have managed a Behcet's disease patient with pathergy test & corticosteroids therapy. We have obtained good result and report this case with review of literatures.

The Pathergy Test in Behcet' s Disease and Recurrent Aphthous Stomatitis / 대한피부과학회지

BACKGROUND: Pathergy, or skin hypersensitivity to needle puncture has been reported as a diagnostic test for Behcets ciisease. But the positivity of pathergy test in recurrent aphthous stomatitis which can be a comporent of Behcets disease is obscure. In recent years it has been noted that the positivity of the test has decreased. It is possible to say that the disposable needles used nowadays are less trauniatic to initiate the reaction than are the nondisposable ones used in the preAIDS ara by some uthors. OBJECTIVE: It is the porpose of this study to clarify the value of pathergy test in differentiating Behcets disease from recurrent aphthous stomatitis and to evaluate the difference of positivity using various kinds of needles, and finally to suggest the most profitable one. METHODS: 34 patients with Behcets disease according to the diagnostic criteria of Shimizu(revisecl) 25 ones in case of applying that of International study group for Behcets disease(ISG) and 21 ones with recurrent aphthous stomatitis, and 25 normal controls were selected. A pathergy test, i.e., intradermal injection of 0.1 ml physiological saline by using 26 gauge & 20 gauge disposable needles and 26 gauge & 20 gauge nondisposable ones were given and the evaluation was made after 24 hours and 48 hours. RESULTS: 1. The positive rates of pathergy test using all kinds of needles in Behcets disease were significantly higher than thoen recurrent aphthous stomatitis and normal controls(p=0.00000-0.03600). The sensitivity were 29.4% 64.7% (Shimizu) and 32.0% 76.0% (ISG), respectively, and the specificity were 91.3%-100.0% for all of them. 2. In Behcets disease, the positive rates of the pathergy test using 26 gauge disposable needles were not significantly different from that of using ZO gauge disposable needles in total and active patients after 24 and 4.3 hours and so were 26 gauge R 20 gauge nondisposable ones(p=0.40134 -0.80603(Shimizu), 0.37551-0.77078(ISG) ). 3. In Behcets disease, the positive rates of pathergy test using 26 gauge nondisposable needles were 59%-68%(Shimiu) and 64% 79% (ISG), which were significantly different, from the test using 20 gauge disposable needles, i.e. 29%-36%(Shimizu), 40%-46%(ISG) in total and active patients(p==0.00753-0.01512) after 24 and 48 hours except for those by the criteria of ISG after 24 hours(p=0.14573-0.15597). CONCLUSION: This study shows that the pathergy test is obviously useful in differentiating Behcets disease from ecurrent aphthous stomatitis and we recommend the intradermal injection of 0.1 ml physiological saline by using 26 gauge nondisposable needle and the evaluation after 48 hours as a standard mithod of pathergy test.

Clinical and histopathologic findings of pathergy test sites in patients with Behcet's disease / 대한피부과학회지

Through the clinicopathologic study of pathergy test sites of 16 patients with Behrets disease in the active or inactive stage, the following results were obtained; 1. In the active stage, six of 16 patients (37.5%) showed positive reactions in clinical pathergy tests, which are much higher than that in the inactive stage when only one patient showed a positive reaction. The severity of the ciniral pathergy reaction was directly related to the activity of the disease. 2. The characteristic histopathologic finding in the active stag was a dermal inflammatory cellular infiltration composed mainly of polymorphonuclear lukocytes and of lymphomononuclear cells accompanied by leukoctocylasia. Two of 16 patients showed true leukocytoclastic vasculitis. 3. Although the type of disease and the reactivity of the clinical pathergy test were related in terms of the severity of the histopathologic findings, there were some notible histopathologic changes even in the cases with negative clinical pathergy reactions. We concluded that the histopathologic findings of the pathergy test sites were more reliable for the diagnosis of Behcet's disease than the clinical findinigs.