Multiple Symmetric Lipomatosis of Madelung-Launois-Bensaude with Alcoholic Liver Cirrhosis: Report of a Case

酒精性肝硬化併多發對稱性脂肪瘤：一病例報告

連昭明(Jau-Min Lien) ； 陳澤卿(Tse-Ching Chen) ； 邱正堂(Cheng-Tang Chiu)

《中華民國消化系醫學雜誌》 14卷3期 (1997/09) Pp. 269-276

https://doi.org/10.6557/GJTb.199709_14(3).0007

摘要

Madelung-Launois-Bensaude多發對稱性脂防瘤為酒精性肝病的一個罕見的表徵。這個症候群常見於地中海地區，多為中年男性並有過量飲酒病史的病人。病人會產生頸部，肩膀及上軀幹多發但兩側對稱的脂肪瘤，給予一類似庫欣氏症候群的表徵。它產生的原因，被認為在於脂肪組織之脂肪分解缺陷與脂蛋白分解酶活性增加有關。臨床上，偶會有周邊與自主神經病變及對胸中隔的侵入。這個症候群必須與庫欣氏與酒精性假庫欣氏症候群區分。其餘頸部病變很容易由電腦斷層作鑑別診斷。我們這裡報告一例56歲的酒精性肝硬化病人併發多發對稱性脂肪瘤診斷及追蹤的經驗。

關鍵字

多發對稱性脂肪瘤 ； 庫欣氏症候群

並列摘要

Multiple symmetric lipomatosis of Madelung-Launois-Bensaude is a rare disease and has been well described in Mediterranean regions, affecting mainly adult Caucasian males with a history of excessive alcohol intake. Patients present with symmetric growth of lipomatous tissues in neck and shoulders which slowly progress to involve upper trunk and back, thus taking a distinct Cushingoid appearance. Its pathogenesis may be related to impaired lipid metabolism of adipose tissues with increased lipoprotein lipase activity and insensitivity to epinephrine stimulated lipolysis. Its clinical course is mostly benign, but may occasionally lead to somatic and autonomic neuropathy. Differential diagnosis will include Cushing's syndrome and alcohol-induced pseudo-Cushing's syndrome. Here we report a case of this syndrome in a 56 year-old male with biopsy proven alcoholic liver cirrhosis who has been followed in our clinic for two years. Literature review is provided.

並列關鍵字

Multiple symmetric lipomatosis ； Cushing's syndrome