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內科學誌 Scopus

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篇名 不只是皮膚疾病:以內科醫師觀點談毒性表皮溶解症
卷期 21:1
並列篇名 Not Just A Dermatologic Disease: Toxic Epidermal Necrolysis from The Viewpoint of An Internist
作者 黃立言劉秉一鄭幼文張俊梁諶立中
頁次 012-025
關鍵字 毒性表皮溶解凋亡顆粒溶解素多重器官衰竭敗血症 ScopusTSCI
出刊日期 201002

中文摘要

毒性表皮溶解症(TEN)是一個少見但會危及生命的疾病,特徵是廣泛性的表皮脫落與黏膜糜爛。在病灶處的皮膚切片可以見到顯著的角質細胞凋亡現象。毒性表皮溶解症通常是藥物所引起,而在台灣,常見的致病藥物包括了抗癲癇藥物、抗生素、降尿酸藥物allopurinol 與非類固醇抗發炎藥物。毒性表皮溶解症的病理生理機轉目前尚未明瞭,現在認為複雜的免疫反應扮演相當重要的角色。毒性表皮溶解症不只是一個皮膚科疾病,因為許多器官會因為發炎性細胞激素的釋放而受到影響。因此內科醫師在治療這些病人方面佔有關鍵性的地位。目前對於毒性表皮溶解症除了儘早停用治病藥物與重症支持性照顧外並沒有廣為接受的特殊療法。敗血症與多重器官衰竭是造成病人死亡的主因。SCROTEN是一套以七個獨立危險因子來評估毒性表皮溶解症病人病情嚴重度與預測死亡率的準確評分系統。希望日後能夠有更進一步研究結果使得我們對於毒性表皮溶解症的致病原理能

英文摘要

Toxic epidermal necrolysis (TEN) is a rare but life-threatening disease characterized by extensive detachment of the epidermis and mucous membrane erosions. Markedkeratinocyte apoptosis is seen in the
affected skin biopsy. TEN is usually drug-related, and common causative drugs in Taiwan include anticonvulsants,antibiotics, allopurinol and nonsteroidal anti-inflammatory drugs (NSAIDs). Until recently, the pathophysiology of TEN was not well understood; now, it is believed that complex immune responses play an important role. TEN is not just a dermatological disease because many organs may be involved due to the release of inflammatory cytokines. Thus, internists play a crucial role in treating these patients. There is no generally accepted specific
treatment except early withdrawal of the causative drugs and intensive supportive therapy. Sepsis and multiple organ failure are major causes of death. SCORTEN is an accurate scoring system that measures 7 independent
risk factors and thus can be used to estimate severity and to predict mortality among TEN patients. Further studies are expected to enhance understanding of the pathogenesis and will lead to development of new
therapeutic agents for this devastating disorder.

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