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題 名 | A Rare Clinical Presentation of Cryofibrinogenemia--Extensive Perineal Skin Necrosis=臨床罕見的冷凍纖維蛋白原血症病例報告--廣泛性會陰部皮膚壞死 |
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作 者 | 蔡明峰; 張世幸; 董光義; 張耀元; 黃文成; 蕭弘道; | 書刊名 | 臺灣整形外科醫學會雜誌 |
卷 期 | 21:3 2012.09[民101.09] |
頁 次 | 頁265-272 |
分類號 | 415.74 |
關鍵詞 | 冷凍纖維蛋白原血症; 皮膚缺血性病變; 會陰部潰瘍; Cryofibrinogenemia; Fournier's gangrene; Perineal ulceration; |
語 文 | 英文(English) |
中文摘要 | 背景:冷凍纖維蛋白原血症(cryofibrinogenemia)雖然具有致命性,在臨床上卻常被低估。即便是已經有許多治療方式被提出來研究,然而這些治療方式的療效仍然並不明確。目的及目標:我們將報告一位八十歲男性在冬季重覆發生類似佛尼爾氏壞疽(Fournier's gangrene) 的會陰部潰瘍,而最終的診斷則是冷凍纖維蛋白原血症。材料及方法:這位病患在幾次的清創及補皮手術之後,在最近的一次發作中,甚至進展成為大片的大腿及雙腳皮膚壞死。因此我們在陰囊壞死的邊緣處做了皮膚的切片,用以鑑別診斷。結果:組織病理檢查中,我們發現在淺層及深層的血管叢雖然沒有鈣質沉積,卻都表現出血栓性的血管病變。在攝氏4度下,冷凍沉澱物質(Cryoprecipitate)出現在檸檬酸化的血液檢體中。這樣的報告證實了冷凍纖維蛋白原血症的診斷。結論:冷凍纖維蛋白原血症會造成許多不同方式表現的皮膚缺血性病變,當有無法解釋的反覆性皮膚缺血性病變時必須將之列為鑑別診斷之一。 |
英文摘要 | Background: Cryofibrinogenemia, a thrombotic vascular disease that is relatively uncommon is an underestimated disease, but can be life-threatening. There are a wide range of treatment choices currently available, including high dose steroids, stanazolol, and certain immunosuppressants, but to date no effective treatment has been established. Aim and Objectives: We are reporting on an 80-year-old male patient who presented with a recurrent Fournier's gangrene-like perineal ulceration during the winter season. The final diagnosis was cryofibrinogenemia. Material and Methods: The study focuses on a recurrent large area of skin necrosis over his thighs and gangrene change of his feet developed after several times of debridement and skin grafting. A skin biopsy sample was taken from the edge of the scrotal necrosis. Results: The histopathologic findings showed thrombotic vasculopathy in both superficial and deep plexus without calcium deposition. Cryoprecipitate was present in the citrated blood samples under 4°C. The reports revealed cryofibrinogenemia. Conclusion: Cryofibrinogenemia may cause various cutaneous ischemic changes, and the differential diagnosis of cryofibrinogenemia should be included in cases of unexplainable recurrent skin ischemic changes. |
本系統中英文摘要資訊取自各篇刊載內容。