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Severe Oral Ulcers in a Patient with Atypical Behçet's Disease - A Case Report

嚴重口腔潰瘍之非典型貝西氏症患者-病例報告

摘要


貝西氏病是罕見且原因不明的免疫相關系統性疾病,主要症狀有復發性口腔潰瘍、生殖器潰瘍、皮膚病灶、和眼睛炎症。本病例是一位29歲女性因腸胃道出血住院。依據其臨床表現曾一度懷疑有罹患鼻型淋巴結外的自然殺手/T細胞淋巴癌的可能性,但經過一連串相關的檢查後診斷為非典型之貝西氏病。住院治療期間施予抗生素、抗發炎藥物與支持性治療,而症狀獲得緩解,並於入院十一天後出院。出院一個月後的定期回診,病人口腔潰瘍復發,持續施予支持性治療與口腔清潔維護。藉此病例與臨床醫師分享貝西氏病之治療經驗,以及鼻型淋巴結外的自然殺手/T細胞淋巴癌以類似貝西氏病的臨床特徵表現與貝西氏病的鑑別診斷。

並列摘要


Behçet's disease (BD) is a rare immune-mediated, small-vessel systemic vasculitis that presents with mucous membrane ulceration and ocular inflammation. We describe a 29-year-old female who was previously admitted for gastrointestinal tract bleeding and diagnosed with BD with atypical symptoms 1 year before visiting the division of rheumatology in consultation with our clinic. Subsequently, the patient regularly revisited the outpatient department. During hospitalization, the differential diagnosis of BD and nasal-type extranodal natural-killer/T-cell lymphoma (ENK/TL) was considered. The final diagnosis was BD, and the patient received anti-inflammatory agents and supportive therapy for 11 days of hospitalization and was discharged. We present this case to demonstrate the management strategy for BD and the possibility of nasal-type ENK/TL mimicking BD.

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