Toxic epidermal necrolysis (TEN) is a type of severe skin reaction. Together with Stevens–Johnson syndrome (SJS) it forms a spectrum of disease, with TEN being more severe. Early symptoms include fever and flu-like symptoms. A few days later the skin begins to blister and peel forming painful raw areas. Mucous membranes, such as the mouth, are also typically involved. Complications include dehydration, sepsis, pneumonia, and multiple organ failure.
Stevens-Johnson Syndrome (SJS) 與 Toxic Epidermal Necrolysis (TEN) 為皮膚大面積壞死與脫落的罕見疾病。 在治療上,環孢素對 SJS 效果顯著;而靜脈注射免疫球蛋白 (IVIg) 與皮質類固醇的合併使用,對 SJS 與 TEN 病例的療效最佳。 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare diseases that are characterized by widespread epidermal necrosis and sloughing of skin. Regarding treatment, cyclosporine is the most effective therapy for the treatment of SJS, and a combination of intravenous immunoglobulin (IVIg) and corticosteroids is most effective for SJS/TEN overlap and TEN.
Toxic epidermal necrolysis (TEN) 是由某些藥物或免疫系統異常活動引發的嚴重皮膚反應,會導致表皮大規模脫落,累及身體表面超過 30%。 TEN 的死亡率超過 20%,主要因感染與呼吸困難而致。停用致病藥物、提供支持性護理,並加以適當的額外治療,可改善預後。近期研究(基於隨機對照試驗及多項分析)顯示,環孢素、腫瘤壞死因子α抑制劑,以及靜脈注射免疫球蛋白與皮質類固醇的組合等藥物,可能對治療 TEN 有幫助。 Toxic epidermal necrolysis (TEN) is a serious skin reaction caused by certain medications and immune system activity, resulting in large-scale detachment of the outer skin layer (epidermis), affecting more than 30% of the body's surface. TEN has a mortality rate of over 20%, often due to infections and breathing difficulties. Stopping the medication causing the reaction, providing supportive care, and using additional treatments can improve the outcome. Recent studies have shown that drugs like cyclosporine, tumor necrosis factor alpha inhibitors, and a combination of intravenous immune globulin and corticosteroids can be helpful, based on randomized controlled trials and analyses of multiple studies.
Recent Advances: There is improved understanding of pain and morbidity with regard to the type and frequency of dressing changes. More modern dressings, such as nanocrystalline, are currently favored as they may be kept in situ for longer periods. The most recent evidence on systemic agents, such as corticosteroids and cyclosporine, and novel treatments, are also discussed.
最常見的誘因是特定藥物,例如 lamotrigine(拉莫三嗪)、carbamazepine(卡馬西平)、allopurinol(別嘌呤醇)、sulfonamide antibiotics(磺胺類抗生素)以及 nafarelin(奈韋拉平)。危險因子包括 HIV 感染與系統性紅斑狼瘡。治療通常需住院,於燒傷科或加護病房進行。
○ 治療
此病危重,若出現口唇或口腔受累、皮膚起水泡,請立即就醫。應立即停用可疑藥物(如 antibiotics(抗生素)、NSAIDs(非類固醇抗發炎藥)等)。