不只是皮膚疾病：以內科醫師觀點談毒性表皮溶解症

Not Just a Dermatologic Disease: Toxic Epidermal Necrolysis from the Viewpoint of an Internist

黃立言(Li-Yen Huang) ； 劉秉一(Bing-Yi Liu) ； 鄭幼文(Yoiu-Wen Cheng) ； 張俊梁(Junn-Liang Chang) ； 諶立中(Lih-Jong Shen) ； 許文欣(Wen-Hsin Hsu) ； 何裕鈞(Yuh-Juin Ho) ； 柯朝元(Chou-Yuan Ko) ； 柯富彰(Fu-Chang Ko)

《內科學誌》 21卷1期 (2010/02) Pp. 12-25

https://doi.org/10.6314/JIMT.2010.21(1).03

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，現在認爲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，因爲許多器官會因爲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爲接受的特殊療法。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有更多的瞭解，並能藉此研發出新的治療藥物，來有效治療這個對人體破壞力強大的疾病。

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

並列摘要

Toxic epidermal necrolysis (TEN) is a rare but life-threatening disease characterized by extensive detachment of the epidermis and mucous membrane erosions. Marked keratinocyte 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.

並列關鍵字

Toxic epidermal necrolysis, TEN ； Apoptosis ； Granulysin ； Multiple organ failure ； Sepsis