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Behçet Disease with Initial Presentation of Vulvar Ulcers: Two Case Reports

以外陰潰瘍為初表現之貝賽特氏症-病例報告

摘要


Objectives: Behçet's disease is a heterogeneous pathological entity of unknown etiology affecting many organs concurrently. Case Report: We reported two cases with ulceration presentations of the vulvar area as the first symptom, highlighting the diagnostic difficulties until Behçet's disease was confirmed. The first patient suffered from right vulvar ulceration for days. She was admitted to our hospital because of frequent diarrhea and lower abdominal pain. Her colonoscopy demonstrated a diffused involvement of multiple ulcers with inflammatory polyps. The second young female patient had right vulvar ulcers and oral ulcerations off and on for several months. Besides, symptoms including fever, and abdominal pain were complained. Supportive treatment was given to both of the patients. Conclusion: Consideration of Behçet's disease is suggested as a possible differential diagnosis of vulvar ulcers.

並列摘要


貝賽特氏症Behçet' sdisease/貝切特症候群Behçet's syndrome貝賽特氏症的患者大多數病例發生在東亞,中東及地中海地區。這是一種病因尚未完全清楚的全身性免疫系統疾病,同時也是一種罕見的微血管血管炎,可能因基因問題引起免疫異常而致病。貝賽特氏症典型的三大臨床特徵是1.口腔潰瘍2.生殖器潰瘍及3.眼睛葡萄膜/網膜炎。同時可能侵犯消化道,肺臟,肌肉骨骼系統,中樞神經系統等等器官的血管造成發炎,嚴重的甚至可能危及生命。許多患者都會有反覆性的口腔潰瘍(recurrent oral ulcers),大約有8成患者會發生生殖器部位的潰瘍(recurrent genital ulcers),女性常發生在大小陰唇外陰等處。陰道潰瘍大多不甚疼痛,但是容易反覆發作而且殘留疤痕。本病例報告指出兩例於本院就診並且以生殖道潰瘍為首發症狀之病患,提醒臨床醫師在眾多生殖道疾病診斷就診時勿忘記這少見的疾病,尤其是得到及時診斷治療後避免其他腸道與眼睛的症狀發生。即便發現臨床症狀也可以透過治療緩解。病情反覆症狀復發為貝賽特氏症的重要特徵,此病症難以預防,長期追蹤及藥物選擇及調整是非常必要的。此外,為免延誤病情,早期診斷也甚重要。有反覆口腔或會陰潰瘍或是眼球葡萄膜炎者,宜僅早至門診做詳細診療。

並列關鍵字

外陰 生殖器潰瘍 貝賽特氏症

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