原發性膽汁鬱積性肝硬化之皮膚表徵：著重於黃色瘤之表現-21例病例研究

Skin Manifestations in Primary Biliary Cirrhosis: with Emphasis on Xanthomas-A Study of 21 Cases

楊志雄(Chih-Hsiung Yang) ； 朱家瑜(Chia-Yu Chu)

《中華皮膚科醫學雜誌》 21卷4期 (2003/12) Pp. 304-316

https://doi.org/10.29784/DS.200312.0001

摘要

我們在此回顧過去12年來本院診斷為原發性膽汁鬱積性肝硬化的21例病例之皮膚表現，其中以搔癢(61.9%)最常見，其次分別是黃疸(52.4%)、黃色瘤與眼瞼黃斑瘤(33.3%)、色素過度沉著症(14.3%)、扁平苔蘚(4.8%)。同時也在此報告三個原發性膽汁鬱積性肝硬化併多樣性黃色瘤的表現，藉此釐清此類病人脂蛋白代謝異常與各型黃色瘤的關係；進而使黃色瘤成為診斷此類病患脂質異常的利器。用飲食控制、藥物及血液透析，皆不能改善其大小及數目；唯有肝臟移植是徹底且成功的治療方法。(中華皮誌21:304-316，2003)

並列摘要

We herein retrospectively analyze the cutaneous manifestations of 21 cases with primary biliary cirrhosis(PBC) during the past 12 years in our hospital. Among them, the most common skin manifestations of PBC is pruritus (61.9%), followed by jaundice (52.4%), xanthomas and xanthelasmas (33.3%), hyperpigmentation (14.3%), and lichen planus (4.8%). We also described three cases presenting as several types of xanthomas due to secondary hypercholesterolemia and hyperlipidemia in PBC. The relationship between lipoprotein disturbances and different types of xanthomas was identified. Types of xanthomas are valuable diagnostic markers of the underlying lipid and lipoprotein disturbances. In these patients, despite conventional dietary, drug, and plasmapheresis therapies, there was no improvement in the size and number of the xanthomas. Liver transplantation for decompensated chronic liver disease resulted in complete resolution of xanthomas in all our cases. It is a drastic but successful remedy for complications of abnormal lipid metabolism associated with primary biliary cirrhosis.(Dermatol Sinica 21 : 304-316,2003)

並列關鍵字

Xanthoma ； Primary biliary cirrhosis ； Liver transplantation