Toxic epidermal necrolysis and Stevens-Johnson syndrome.
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
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DOI：
10.1186/1750-1172-5-39
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发表时间：
2010-12-16
影响因子：
3.7
通讯作者：
French LE
中科院分区：
文献类型：
--
作者：
Harr T;French LE
中毒性表皮坏死松解症（TEN）和史蒂文斯约翰逊综合征（SJS）是严重的皮肤药物不良反应，主要累及皮肤和粘膜。这两种情况都很罕见，每年大约有1 / 2/ 100万人受到TEN和SJS的影响，它们被认为是医疗紧急情况，因为它们可能致命。它们的特征是皮肤粘膜压痛，典型的出血性糜烂，红斑和或多或少严重的表皮脱离，表现为水泡和皮肤脱落。目前，TEN和SJS被认为是严重表皮松解性皮肤药物不良反应的两个极端，它们的区别只是皮肤脱离的程度。在大多数情况下，药物被认为或确定为SJS/TEN的主要原因，但肺炎支原体和单纯疱疹病毒感染是有充分记录的原因，还有一些病因不明的罕见病例。几种药物具有诱发TEN/SJS的“高”风险，包括：别嘌呤醇、甲氧苄啶-磺胺甲恶唑和其他磺胺类抗生素、氨基霉素、头孢菌素、喹诺酮类、卡马西平、苯妥英、苯巴比妥和奥昔康类非甾体抗炎药。在汉族人群中，人类白细胞抗原HLA-B*1502与卡马西平诱导的SJS有很强的相关性，这可能是SJS和TEN的遗传易感性。诊断主要依靠临床症状和皮肤活检的组织学分析，显示典型的全层表皮坏死松解，原因是广泛的角化细胞凋亡。鉴别诊断包括线性IgA皮肤病和副肿瘤性天疱疮、寻常型天疱疮和大疱性类天疱疮、急性全身性脓疱病（AGEP）、弥漫性固定大疱性药疹和葡萄球菌性烫伤皮肤综合征（SSSS）。由于死亡率高，SJS/TEN患者的管理需要快速诊断，使用SCORTEN评估预后，识别和中断罪魁祸首药物，最好在重症监护病房进行专门的支持治疗，并考虑使用免疫调节剂，如大剂量静脉注射免疫球蛋白治疗。SJS和TEN病情严重，危及生命。报告的SJS平均死亡率为1-5%，TEN为25-35%；在老年患者和表皮脱落面积较大的患者中，这一比例甚至更高。超过50%存活下来的患者患有这种疾病的长期后遗症。
Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. Both are rare, with TEN and SJS affecting approximately 1or 2/1,000,000 annually, and are considered medical emergencies as they are potentially fatal. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Currently, TEN and SJS are considered to be two ends of a spectrum of severe epidermolytic adverse cutaneous drug reactions, differing only by their extent of skin detachment. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. Diagnosis relies mainly on clinical signs together with the histological analysis of a skin biopsy showing typical full-thickness epidermal necrolysis due to extensive keratinocyte apoptosis. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP), disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS). Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, evaluation of the prognosis using SCORTEN, identification and interruption of the culprit drug, specialized supportive care ideally in an intensive care unit, and consideration of immunomodulating agents such as high-dose intravenous immunoglobulin therapy. SJS and TEN are severe and life-threatening. The average reported mortality rate of SJS is 1-5%, and of TEN is 25-35%; it can be even higher in elderly patients and those with a large surface area of epidermal detachment. More than 50% of patients surviving TEN suffer from long-term sequelae of the disease.
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DOI：
10.1097/01.bcr.0000105096.93526.27
发表时间：
2004-01-01
期刊：
JOURNAL OF BURN CARE & REHABILITATION
影响因子：
--
作者：
Brown, KM;Silver, GM;Gamelli, RL
通讯作者：
Gamelli, RL
影响因子：
4.1
作者：
Callaly, E. L.;FitzGerald, O.;Rogers, S.
通讯作者：
Rogers, S.
影响因子：
6
作者：
Abe, R;Shimizu, T;Shimizu, H
通讯作者：
Shimizu, H
影响因子：
6.5
作者：
Bastuji-Garin, S;Fouchard, N;Wolkenstein, P
通讯作者：
Wolkenstein, P
影响因子：
82.9
作者：
Chung, Wen-Hung;Hung, Shuen-Iu;Chen, Yuan-Tsong
通讯作者：
Chen, Yuan-Tsong