1/59. A case of pediatric Behcet's disease with intestinal involvement.We report a 6-year-old girl with recurrent oral aphthae, genital and perianal ulcers, and folliculitis-like erythema followed by intestinal symptoms. No ocular lesions were found. Her ailment was diagnosed as incomplete Behcet's disease with intestinal involvement. According to the literature, pediatric Behcet's disease is characterized by a low incidence of ocular lesions and a high incidence of intestinal involvement, as exhibited in this case. As intestinal lesions in pediatric Behcet's disease are often life-threatening, a barium enema must be given to exclude the possibility of intestinal involvement when pediatric patients with Behcet's disease complain of abdominal pain.- - - - - - - - - - ranking = 1keywords = erythema (Clic here for more details about this article) |
2/59. "Neuro-sweet disease": benign recurrent encephalitis with neutrophilic dermatosis.OBJECTIVE: To describe benign recurrent encephalitis in a case of sweet syndrome that also showed clinical features of Behcet disease. CASE REPORT: A 37-year-old Japanese man developed relapsing and remitting encephalitis and mucocutaneous symptoms mimicking Behcet disease. Magnetic resonance images showed at least 5 episodes of transient abnormal signal intensity in various cerebral regions over a period of 5 years. A skin biopsy specimen of the cutaneous edematous erythematous plaques revealed neutrophilic dermatitis compatible with sweet syndrome. HLA typing showed B54, which is frequent in sweet syndrome but rare in Behcet disease. Oral prednisolone therapy (10-60 mg/d) was remarkably effective for the encephalitis as well as for the mucocutaneous symptoms. CONCLUSION: We propose that there is an entity that is like Sweet disease, but with recurrent encephalitis characterized by an association with HLA-B54 and a high responsiveness to corticosteroid therapy, which we have tentatively named neuro-Sweet disease, that is distinct from the classic central nervous system involvement of Behcet disease.- - - - - - - - - - ranking = 1keywords = erythema (Clic here for more details about this article) |
3/59. Encapsulated necrosis associated with Behcet's syndrome.We reported a case of a 45-year-old woman with encapsulated necrosis associated with Behcet's syndrome. The lesions were characterized by multiple, small subcutaneous nodules on the extremities. Histologically, a nodule consisted of degenerative adipocytes encapsulated by fibrous tissue. A membranocystic lesion was also observed in the cavity of the nodule. The patient had a long-term history of Behcet's syndrome and suffered from erythematous subcutaneous nodules, resembling erythema nodosum on the extremities with a tendency to recurrence. These observations indicate that Behcet's syndrome could be implicated in the development of encapsulated necrosis as an underlying disease.- - - - - - - - - - ranking = 136.93521212919keywords = erythema nodosum, nodosum, erythema (Clic here for more details about this article) |
4/59. magnetic resonance imaging in the evaluation of patients with aseptic meningoencephalitis and connective tissue disorders.OBJECTIVE: To describe the role of magnetic resonance imaging (MRI) in the evaluation of patients with chronic and recurrent aseptic meningitis. METHOD: A retrospective study of five patients with aseptic meningoencefalitis diagnosed by clinical and CSF findings. CT scans showed without no relevant findings. RESULTS: MRI showed small multifocal lesions hyperintense on T2 weighted images and FLAIR, with mild or no gadolinium enhancement, mainly in periventricular and subcortical regions. meningoencephalitis preceded the diagnosis of the underlying disease in four patients (Behcet's disease or systemic lupus erythematosus). After the introduction of adequate treatment for the rheumatic disease, they did not present further symptoms of aseptic meningoencephalitis. CONCLUSION: Aseptic meningoencephalitis can be an early presentation of an autoimmune disease. It is important to emphasize the role of MRI in the diagnosis and follow-up of these patients.- - - - - - - - - - ranking = 1keywords = erythema (Clic here for more details about this article) |
5/59. Beneficial effects of interferon-alpha in a case with Behcet's disease.At the age of 20 years, a Japanese man with recurrent oral aphthae, genital ulcers, folliculitis, erythema nodosum, episodic arthritis and epididymitis was diagnosed as having Behcet's disease (BD) in 1966. He has had active ocular manifestations of BD since 1990. These symptoms recurred and never abated for a long period of time. A right renal cell carcinoma developed and he underwent right nephrectomy in April 1996. Treatment with interferon-alpha was started from June 1996 as supplemental chemotherapy. No active phase developed during administration of IFN-alpha. We suggest that IFN-alpha may play a role as an immunomodulatory agent in BD.- - - - - - - - - - ranking = 135.93521212919keywords = erythema nodosum, nodosum, erythema (Clic here for more details about this article) |
6/59. Cardiac and great vessel thrombosis in Behcet's disease.Behcet's disease (BD) is a chronic relapsing systemic vasculitis in which orogenital ulceration is a prominent feature. The disease affects many systems and causes hypercoagulability. We present a 27-year-old male patient who exhibited widespread great vessel thrombosis including right atrial and ventricular thrombi in the setting of right-sided infectious endocarditis and orogenital aphthous ulcerations and erythema nodosum due to BD. We reviewed the enigmatic prothrombotic state of BD, and discuss our prior experiences in this field.- - - - - - - - - - ranking = 135.93521212919keywords = erythema nodosum, nodosum, erythema (Clic here for more details about this article) |
7/59. A case of nodular scleritis in association with Behcet's disease.Approximately 50% of patients with scleritis are known to be associated with systemic connective tissue diseases or vasculitic diseases such as rheumatoid arthritis (RA), Wegener's granulomatosis, relapsing polychondritis, and systemic lupus erythematosus. The patients with scleritis in association with Behcet's disease (BD) have been rarely described in the literature. We report a 46-year-old female patient with BD who developed the nodular scleritis.- - - - - - - - - - ranking = 1keywords = erythema (Clic here for more details about this article) |
8/59. Behcet's disease complicated by pylephlebitis and hepatic abscesses.A 22 year old man presented with fever, abdominal pain, weight loss and diarrhea. Past medical history revealed recurrent aseptic meningitis, uveitis, and erythema nodosum. Further inquiry unveiled a prominent history of oral aphthous ulcers; all features of Behcet's disease. Imaging revealed mesenteric arteritis and pylephlebitis, septic thrombophlebitis of the portal vein, a previously unrecognized complication of Behcet's disease, with multiple intrahepatic abscesses. Portal venography demonstrated an extensively diseased, expanded, and obstructed portal venous system. blood cultures and portal vein aspirate yielded polymicrobial flora. Percutaneous intraportal thrombolytic therapy and mechanical thrombectomy were attempted to restore flow to the portal venous system. This distinctly rare manifestation of Behcet's Disease, pylephlebitis, may result from ischemic injury and structural compromise of the bowel mucosa, resulting from underlying vasculitis.- - - - - - - - - - ranking = 135.93521212919keywords = erythema nodosum, nodosum, erythema (Clic here for more details about this article) |
9/59. The incidence and pathology of conjunctival ulceration in Behcet's syndrome.AIMS: To describe the results of a study of the incidence of conjunctival ulceration and its pathology as a manifestation of Behcet's syndrome. methods: The authors reviewed retrospectively medical charts of 152 patients with Behcet's syndrome seen at the uveitis service of Okayama University Hospital from January 1995 to December 1999. Criteria used to diagnose Behcet's syndrome in this study required the presence of oral aphthous ulceration plus any two of three other manifestations-genital ulceration, eye lesions such as iridocyclitis and uveoretinitis, or skin lesions such as erythema nodosum. The presence of conjunctival ulceration was determined in patients with Behcet's syndrome through standardised ophthalmological examinations. A biopsy of the conjunctival ulceration was performed in one patient. The incidence of conjunctival ulceration in patients with Behcet's syndrome was calculated and the clinical and pathological features of this ulceration were examined. RESULTS: Four of 152 patients (2.6%) developed conjunctival ulceration in concordance with exacerbation of their systemic symptoms. Histopathological examinations of a conjunctival biopsy specimen in one patient revealed intraepithelial and perivascular infiltration with neutrophils and lymphocytes, consistent with Behcet's syndrome. The conjunctival ulcerations of the study patients showed good response to topical corticosteroids or topical steroids plus oral colchicine. CONCLUSIONS: Conjunctival ulceration should be noted as an uncommon but possible manifestation of Behcet's syndrome. Accordingly, routine examination of the conjunctiva is recommended in patients with Behcet's syndrome, and Behcet's syndrome should be included in a list of differential diagnoses for patients with conjunctival ulcerations.- - - - - - - - - - ranking = 135.93521212919keywords = erythema nodosum, nodosum, erythema (Clic here for more details about this article) |
10/59. A case of Behcet's disease accompanied by colitis with longitudinal ulcers and granuloma.A 37-year-old female presenting with oral and genital ulcers and erythema nodosum on both arms was diagnosed as having Behcet's disease. The symptoms resolved spontaneously. However, she was admitted to our hospital (Keio University Hospital) several months later because of fever, aphthous ulcers of the oral cavity, lower abdominal pain and frequent diarrhea. A colonoscopic examination revealed multiple ulcers including longitudinal ulcers in the ascending and transverse colon, and histological examination of biopsied specimens demonstrated non-caseating epithelioid granuloma. Treatment with prednisolone and 5-aminosalicylic acid was started, and the patient responded well clinically. One month later, a repeated colonoscopy confirmed that the lesions including longitudinal ulcers had disappeared. In this report, we describe our experience of this rare case of Behcet's disease concomitant with colonic longitudinal ulcers and epithelioid granuloma, and discuss the difficulties in making a differential diagnosis, primarily with regard to Crohn's disease.- - - - - - - - - - ranking = 135.93521212919keywords = erythema nodosum, nodosum, erythema (Clic here for more details about this article) |
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