Cases reported "Behcet Syndrome"

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1/793. Histopathology of Behcet disease. review of the literature with a case report.

    An autopsy case of Behcet disease which was considered to be typical both clinically and histopathologically was reported. Cerebral abscess was also noted. Consequently it was presumed that some of the findings which had been called softening, demyelination, cavitation among others as the lesions of Neuro-Behcet might be closely related to abscess. Besides suppurative endoaortitis which was thought to be of interest as the lesions of Vascular-Behcet was observed. In order to substantiate the above, a general review of the literature of Behcet disease was made. ( info)

2/793. 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. ( info)

3/793. Myelodysplastic syndrome with monosomy 7 after immunosuppressive therapy in Behcet's disease.

    Only few cases of Behcet's and hematological malignancies have been reported until now. We recently observed a 39-year-old female patient with Behcet's disease developing a myelodysplastic syndrome (MDS) FAB subtype refractory anemia with excess of blasts in transformation [RAEB-t] with a monosomy 7 after being treated with cyclosporin A and chlorambucil for several years. This case is reported and the occurrence of hematological malignancies and Behcet's disease is reviewed. ( info)

4/793. pityriasis rosea in a patient with Behcet's disease treated with interferon alpha 2A.

    interferons have been used for the treatment of many diseases because of their immunomodulatory, antiviral, antitumoral, and antiproliferative effects. Systemic interferon alpha 2a (IFN-alpha 2a) is also efficacious in the management of Behcet's disease (BD). Many adverse cutaneous reactions related to IFN-alpha have been reported. In the light of the current literature, we report here the first case of pityriasis rosea (PR) that developed while receiving IFN-alpha 2a for BD. However, since this is the first report on this association, further observations are needed to determine their clinical relevance. ( info)

5/793. thalidomide responsiveness in an infant with Behcet's syndrome.

    BACKGROUND: Behcet's syndrome is a chronic relapsing vasculitis characterized by aphthous stomatitis, genital ulcerations, and uveitis. The disease is a multisystem illness, which may involve the skin, joints, gastrointestinal tract, blood vessels, central nervous system, and other organs. CASE STUDY: We report a case of an infant with features of Behcet's syndrome who did not respond to conventional immunosuppression with high-dose corticosteroids and cytotoxic agents. thalidomide, a potent immune response-modifying drug, was used with marked improvement and resolution of symptoms. CONCLUSION: This finding supports a trial of thalidomide treatment in patients with Behcet's syndrome who are unresponsive to other forms of immunosuppressive therapy or develop undesirable side effects. ( info)

6/793. Peroneal artery aneurysm treated by transcatheter coil embolization and temporary balloon occlusion in Behcet's disease.

    Peroneal artery aneurysms in Behcet's disease have not been described to date. We present such a patient who was treated successfully using transcatheter embolization. ( info)

7/793. Neuro-Behcet's disease presenting with isolated unilateral lateral rectus muscle palsy.

    The authors present the clinical findings of a 30-year-old female and a 29-year-old male who both had isolated unilateral lateral rectus muscle palsy in neuro-Behcet's disease. The clinical feature related to isolated abduscens nerve palsy was identified by CT, systemic assessment and extraocular examination. These patients' constellation of findings appear to be unique: it does not follow any previously reported pattern of ocular manifestations of neuro-Behcet's disease. ( info)

8/793. Demyelination of retinal myelinated nerve fibers in Behcet's disease.

    A 39 year-old Behcet's patient had demyelination of retinal myelinated nerve fibers after recurrent papillitis and vitritis. Oral prednisolone, cyclosporine, and later periocular corticosteroids and oral colchicine were used but demyelination continued over a 5 year-period. A pseudobulbar palsy with urinary incontinence and pyramidal tract signs developed and azathioprine and corticosteroids were used. Demyelination of retinal nerve fibers stopped and while treatment was underway, the central nervous system (CNS) signs were stable. While the ocular pathology of Behcet's can mirror the CNS signs, indeed optic nerve ischemic demyelination may signal the potential for CNS involvement; azathioprine with prednisone may be more effective in the long-term for optic nerve and CNS involvement than cyclosporine with prednisone. ( info)

9/793. Superimposed primary chancre in a patient with Adamantiades-Behcet's disease.

    Adamantiades-Behcet's disease was diagnosed in a 42 year old Turkish patient with recurrent oral aphthae, genital ulcerations, papules, and sterile pustules, histologically presenting as cutaneous vasculitis, and intermittent arthritis with joint effusion particularly of the knees. Six months after initial improvement under treatment with colchicine 2 mg/day, a solitary genital ulcer with enlarged inguinal lymph nodes appeared and persisted for 7 weeks despite the continuation of colchicine treatment and the introduction of clindamycin 2 mg/day intravenously. The unusual persistence of the ulcer and the failure of clindamycin therapy led to further differential diagnostic considerations and the identification of primary syphilis. The genital lesion healed 4 weeks after initiation of treatment with tetracycline 2 mg/day by mouth for 15 days. ( info)

10/793. "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. ( info)
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