良性對稱性脂肪過多病－病例報告

Benign Symmetrical Lipomatosis (Madelung's Disease) – Case Report

陳篤亮(Tu-Liang Chen) ； 劉安邦(An-Bang Liu) ； 王國榮(Kuo-Rong Wang)

《中華民國耳鼻喉科醫學會雜誌》 32卷6期 (1997/12) Pp. 752-758

https://doi.org/10.6286/1997.32.6.752

摘要

背景：良性對稱性脂肪過多病(Benign symmetrical lipomatosis)又稱之為Madelung's disease或Launois-Bensaude syndrome(以下簡稱BSL)，自1846年Brodie首先提出報告以來，至今文獻中大約只有200多個病例。由於此病罕見，且在東方甚少被報告，特收集整理提出報告。
方法：本拜從1989年1月至1997年1月計8年間經歷了5歷BSL，他們均符合此病的診斷標準。本文依病患之臨床資料，飲酒狀況，病理檢查脂肪的分佈與合併出現的全身性疾病等加以分析，並作文獻上的回顧。
結果：5例平均年齡57歲，皆為男性，發病期間平均約5年。5例均無家族史，皆為數十年平均日飲2瓶烈酒的酗酒者。所有的病例均有多量脂肪組織對稱分佈於部與後上背，此外分佈於肩膀及鎖骨上區者各有2例，分佈資腮腺區、枕骨下區、手肘內側、腋下、腰、下腹及下背者各有1例。合併有酒精性肝病、高尿酸血症、痛風及高血壓者各有3例，高三酸甘油脂血症者有2例。5例中有2例接受前頸部腫瘤切除手術，術後追蹤二年半及三年無局部復發現象。
結論：BSL好發於中年男性，病人經常是酗酒者，無家族史。腫瘤無痛，於數年間緩慢生長，主要對稱性分佈於頸部及後上背，此外腮腺區、枕骨下區、軀幹、上及腋下等也有可能涉及，但前的遠端及腿部則無。病人常合併有緎肝病、高尿酸血症、痛風、高血壓及高三酸甘油脂血症。治療方法是減緩性手術切除患部。而只有當病人有嚴重外觀上的變形，心理上的壓力，頸部酸痛，或臨床上有明顯的上呼吸消化道壓迫的情形才有需要開刀。

關鍵字

良性對稱性脂肪過多病 ； Madelung's氏病 ； Launois-Bensaude氏症儘群

並列摘要

Background: Benign symmetrical lipomatosis (Madelung's disease, Launois-Bensaude syndrome) is a rare disease. The first obsrvation of this disease was made by Brodie in 1846. Since then, over 200 cases have been published in the medical literature. Oriental reports about benign symmetrical lipomatosis (BSL) are very rare. Here. we present an article concerning 5 cases of BSL in Taiwan.
Method; From January 1989 to January 1997, we have collected 5 cases of BSL. In this paper we reevaluated the clinical features, association with alcoholism, pathological fat tissue distributions and associated systemic diseases of 5 BSL patients in Taiwan, and review the literature.
Results: The average age of the patients was 57 years. All patients were male. The mean duration of disease was 5 years. There were no evidence of hereditary predispsition. The patients had been heavy drinkers for several decades. Among the 5 patients, there were 5 cases of pathological fat tissue located in the cevical and upper dorsal back, 2 in hte shoulders and 2 in the supraclavicular area; and there were single cases of pathological fat tissue located in the parotid area, suboccipital area, medial elows, axillae, lumbar area, lower abdomen, and lower back respectively. Among the 5 patients, 3 also presented with hyperuricemia, gout, alcohol liver disease and hypertension respectively, and 2 with hypertriglyceriemia. 2 of our cases received surgical excision of anterior neck tumors two and a half years and three years ago respectively; so far, no local recurrene has been noted.
Conclusions: The disease affects almost exclusively middle-aged men, usually with a background of excessive alcohol intake or liver disease, and there is no evidence of hereditary predisposition. The fatty deposits grow insidiously, ofter over many years, and are nontender and painless. They are chiefly located symmetrically in the region of the neck and upper dorsal back but also may involve the parotid area, suboccipital area, trunk, upper arms and axillae. the distal portions of the forearm and leg remain unaffected. The disease is associated with alcohol liver disease, hyperuricemia, gout, hypertension and hypertriglyceridemia. Palliative surgical removal of pathological fat tissue is the recommended treatment, which is performed only if the patient reports severe cosmetic deformity, psychological stress, neck soreness, ork if clinically significant compression of the upper aerodigestive tract occurs.

並列關鍵字

Benign symmetrical lipomatosis ； Madelung's disease ； Launois-Bensaude syndrome