Chamuleau R A, Diekman M J, Bos P J, Smitt J H, Bosma A, Schellekens P T
Dept. of Internal Medicine, University of Amsterdam, The Netherlands.
Hepatogastroenterology. 1992 Dec;39(6):523-4.
A 36-year-old Surinam woman with a severe form of toxic epidermal necrolysis of unknown origin is presented. Skin lesions healed gradually without scarring within 3 to 4 weeks, but eye lesions progressed to symblepharon and corneal opacification, resulting in almost complete blindness. In addition, toxic epidermal necrolysis was associated with severe intrahepatic cholestasis caused by vanished bile duct syndrome; viral hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis were excluded. After about six months, intrahepatic chole-stasis improved spontaneously and a third liver biopsy taken after 51 weeks of illness revealed that the bile ducts had reappeared. At present, the patient is relatively well, with no jaundice, although parameters of cholestasis are still elevated: Alkaline phosphatase three times, and gamma GT thirty times the normal values. This sequence of events has to our knowledge never been reported in the literature.
本文报告了一名36岁的苏里南女性,患有严重的、病因不明的中毒性表皮坏死松解症。皮肤病变在3至4周内逐渐愈合且无瘢痕形成,但眼部病变进展为睑球粘连和角膜混浊,导致几乎完全失明。此外,中毒性表皮坏死松解症与消失胆管综合征引起的严重肝内胆汁淤积有关;排除了病毒性肝炎、原发性胆汁性肝硬化和原发性硬化性胆管炎。大约六个月后,肝内胆汁淤积自发改善,病程51周后进行的第三次肝脏活检显示胆管重新出现。目前,患者情况相对良好,无黄疸,尽管胆汁淤积参数仍升高:碱性磷酸酶为正常值的三倍,γ-谷氨酰转移酶为正常值的三十倍。据我们所知,这一系列事件在文献中从未有过报道。