索拉非尼致重症药疹1例并文献复习

<正>索拉非尼是一种口服的多激酶抑制剂,可抑制肿瘤细胞增殖和肿瘤血管形成。临床用于治疗肝细胞癌、肾细胞癌和甲状腺癌。手足皮肤反应是其常见不良反应,但导致重症药疹如Stevens-Johnson综合征(Stevens-Johnson syndrome, SJS)/中毒性表皮坏死松解症(toxic epidermal necrolysis, TEN)却罕有报道。SJS和TEN被认为是同一系统疾病[1],主要根据体表皮肤剥脱百分比鉴别。     

儿童肺炎支原体诱导的皮疹和黏膜炎临床特征分析

目的 比较肺炎支原体(Mycoplasma pneumoniae,MP)和药物诱导的儿童Stevens-Johnson综合征(Stevens-Johnson syndrome, SJS)的临床特征、治疗管理和预后之间的差异。方法 对河北省儿童医院2010-2021年收治的94例SJS和206例多形性红斑患儿进行筛选,对MP检测阳性的患儿(MP病例组,26例)与MP阴性且有明确药物因素的患儿(药物对照组,31例)进行比较分析。结果 与药物对照组相比,MP病例组患儿以年长儿多见,大于72月龄儿童的占比明显高于药物对照组,且均存在黏膜受累,以眼周黏膜(n=25,96.2%)最常见。其治疗主要为大环内酯类抗生素(100%)、皮质类固醇(84.6%)、免疫球蛋白(26.9%),中毒性表皮坏死松解症严重程度评分(severity-of-illness score for toxic epidermal necrolysis, SCORTEN)在2分及以上,均使用皮质类固醇,应用静脉注射用免疫球蛋白比例随SCORTEN评分增高而升高,MP病例组患儿平均住院日短于药物对照组,未见死亡病例。结论 MP相...     

替雷利珠单抗致中毒性表皮坏死松解症1例报告

目的 了解程序性细胞死亡受体-1（programmed cell death-1,PD-1）/程序性细胞死亡受体-配体1（programmed cell death-ligand 1,PD-L1）抑制剂治疗肿瘤导致Stevens-Johnson综合征（Stevens-Johnson syndrome,SJS）/中毒性表皮坏死松解症（toxic epidermal necrolysis,TEN）的临床特点。方法 回顾性分析海军军医大学第三附属医院皮肤科诊治的1例及既往文献报道的31例PD-1/PD-L1抑制剂治疗肿瘤导致SJS/TEN的临床资料。结果 共收集32例，其中SJS 15例，潜伏期平均8.4周，SJS-TEN 3例，潜伏期平均2.5周，TEN 14例，潜伏期平均3.8周。好转21例，转为慢性扁平苔鲜1例，加重1例，死亡8例，未报道1例。结论PD-1/PD-L1抑制剂治疗肿瘤导致SJS/TEN少见但危重，需引起临床医师的高度重视。     

重症药疹37例临床回顾

药疹是皮肤药物不良反应(skin adverse drug reactions)最常见的表现形式,通常为发疹型,但有时在病程早期即表现为严重的皮肤和黏膜损害例如Steven-Johnson综合征(Steven-Johnson syndrome, SJS)或中毒性表皮坏死松解症(toxic epidermal necrolysis, TEN).重症药疹皮损广泛,伴有全身中毒症状和内脏受累,易出现严重并发症,病死率较高,一般包括SJS、TEN和剥脱性皮炎(exfoliative dermatitis, ED)3种类型. 为了研究重症药疹的临床特征, 现对本科1993年1月～2004年10月收治住院的37例重症药疹患者的临床资料进行分析并报告如下.     

新生儿剥脱性皮炎临床分析

新生儿剥脱性皮炎、又称葡萄菌型中毒性表皮松解坏死症(staphylococcal toxic epidermal necrolysis即TEN)(亦称lyell病)及金葡萄性烫伤样皮肤综合症(staphyloccoccaus scalded skin syndrome，SSSS)，是由凝固酶阳性噬菌体Ⅱ组的金葡萄球菌(71型)引起的婴儿急性表皮颗粒层细胞坏死的严重型皮肤感染。     

不同类型重症药疹患者外周血淋巴细胞亚群分析及其临床意义

目的:检测不同类型重症药疹患者外周血淋巴细胞亚群并研究其临床意义?方法:采用免疫荧光标记流式细胞术检测21例重症药疹患者外周血淋巴细胞的表型?结果:药物超敏反应综合征(drug-induced hypersensitivity syndrome,DIHS)患者治疗前CD3+?CD3+CD4+淋巴细胞较正常值显著增加,并且DIHS患者治疗前CD3+CD4+淋巴细胞显著高于Stevens-Johnson综合征(Stevens-Johnson syndrome,SJS)和中毒性表皮坏死松解症(toxic epidermal necrolysis,TEN)患者;在SJS和TEN患者中,随着外周血CD3+CD4+?CD3+CD8+淋巴细胞数目的增加,激素最大控制用量逐渐减少,随着外周血CD3-CD16+CD56+淋巴细胞数目的增加,激素最大控制用量逐渐增加?结论:不同类型的重症药疹外周血淋巴细胞亚群不同,外周血不同类型淋巴细胞亚群对激素最大控制用量的影响不同?     

中毒性表皮坏死松解型药疹的救治体会

中毒性表皮坏死松解型药疹（toxic epidermal necrolysis，TEN）是病情最急，病势最凶的皮肤药物反应之一。其特征为快速而广泛的表皮剥脱伴全层表皮坏死，并常有多脏器损害。本科在2002年8月。2004年9月连续抢救了8例中毒性表皮坏死松解型重型药疹，现报告如下。     

糖皮质激素单用或联合丙种球蛋白治疗SJS/TEN的临床疗效:基于SCORTEN评分的回顾性分析

目的 评估SCORTEN评分与我国SJS/TEN患者疾病严重程度的一致性,比较单用糖皮质激素(TCS)和糖皮质激素—丙种球蛋白冲击(TCS-IVIG)联合治疗SJS/TEN的临床效果.方法 收集我院2005年6月—2015年5月住院的SJS/TEN患者,计算每例患者的SCORTEN评分,采用Hosmer-Lemeshow检验评估SCORTEN模型的预期死亡率和实际死亡率的拟合度;比较单用TCS和TCS-IVIG联合治疗的患者在疾病严重程度、 住院天数、 疾病控制时间和死亡率方面的差异.结果 SCORTEN模型的预期死亡率和实际死亡率之间的拟合度良好(各组P值均大于0.5);二组患者在住院天数、 疾病控制时间和死亡率方面的差异没有统计学意义(P分别为0.105,0.910,0.701),但TCS-IVIG组患者的疾病严重程度显著高于TCS组(P=0.017).结论 SCORTEN评分可以用于评估国内SJS/TEN患者的病情严重程度,并预测患者预后;与单用TCS相比,联合IVIG有助于提高重症SJS/TEN患者的救治效果.     

重症药疹临床分析

重症药疹(severe drug eruption,SDE),主要包括剥脱性皮炎型(exfdiative dermatitis,ED),重症多形红斑型(erythema multiforme major,EMM)及中毒性表皮坏死松解型(toxic epidermal necrolysis,TEN)。特点为起病急骤,进展迅速,皮损广泛,全身中毒症状严重,多伴发黏膜及多脏器受累,易出现各种并发症,死亡率高,现对我院2001年～2012年收治的39例重症药疹的临床资料进行回顾性分     

卡马西平诱发药疹与HLA-B在汉族人群中的关系

Stevens-Johnson综合征(SJS)和中毒性表皮坏死松解症(TEN)是一类发病率低却可危及生命的皮肤不良反应,卡马西平(CBZ)是常见诱发药物之一。近年来许多地区报道HLA-B*1502与CBZ在汉族人群中诱发SJS和TEN强相关,而在高加索人种中却没有类似发现,存在种族差异。目前CBZ仍然是临床中常用药物。现对CBZ诱发SJS和TEN与HLA-B*1502在汉族人群的关系进行综述,为临床用药安全性提供参考。     

Adefovir-induced Stevens-Johnson syndrome and toxic epidermal necrolysis overlap syndrome

An increasing number of patients with chronic hepatitis B infection are being treated with the newly licensed drug, adefovir. It is an acyclic nucleoside phosphonate that is relatively safe in the dosage generally used for chronic hepatitis B. Serious adverse cutaneous drug reactions like Stevens-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) following adefovir use have not been reported. We report a case of adefovir-induced SJS and TEN overlap syndrome in a patient with chronic hepatitis B infection.     

继发呼吸道烟曲霉菌感染的中毒性表皮坏死松解型药疹1例

Among the various drug induced dermatological entities toxic epidermalnecrolysis (TEN) and Stevens-Johnson syndrome (SJS) occupy a primary place in terms of mortality. Toxic epidermal necrolysis also known as Lyell’s syndrome was first described by Lyell in 1956. Drugs are by far the most common cause of toxic epidermal necrolysis, in which large sheets of skin are lost from the body surface making redundant the barrier function of the skin, with its resultant complications. Drug-induced toxic epidermal necrolysis are severe adverse cutaneous drug reactions to various precipitating agents that predominantly involve the skin and mucous membranes. Toxic epidermal necrolysis is rare but considered medical emergencies as they are potentially fatal. Drugs are the most common cause accounting for about 65%-80% of the cases. The most common offending agents are sulfonamides, NSAIDs, butazones and hydrantoins. An immune mechanism is implicated in the pathogenesis, but its nature is still unclear. There is a prodormal phase in which there is burning sensation all over the skin and conjunctivae, along with skin tenderness, fever, malaise and arthralgias. Early sites of cutaneous involvement are the presternal region of the trunk and the face, but also the palms and soles, rapidly spread to their maximum extent, the oral mucosa and conjunctiva being affected. Initial lesions are macular, followed by desquamateion, or may be from atypical targets with purpuriccenters that coalesce, from bullae, then slough. The earlier a causative agent is withdrawn the better is the prognosis. Several treatment modalities given in addition to supportive care are reported in the literature, such as systemicsteroids, high-dose intravenous immunoglobulins, ciclosporin, TNF antagonists. Recovery is slow over a period of 14-28 days and relapses are frequent. Mortality is 25%-50% and half the deaths occur due to secondary infection. Here we report a 50-year-old female of drug-induced toxic epidermal necrolysis. She was admitted to the dermatology ward with extensive peeling of skin over the trunk and limbs. She had taken alamotrigine for epilepsy. A week after taking the tablets, the patient developed a severe burning sensation all over the body and followed by a polymorphic erythematous dermatitis and widespread peeling of skin. We treated this patient with high dose corticosteroids, high-dose intravenous immunoglobulins and etanercept, but eventually she died of secondary aspergillus fumigatus infection.     

重症多形红斑及中毒性表皮坏死松解症73例临床分析

目的 分析重症多形红斑(SJS)和中毒性表皮坏死松解症(TEN)的致病因素、临床特征及治疗与转归，为其临床防治提供依据。 方法 回顾性分析杭州市第三人民医院收治的73例SJS和TEN患者的临床资料，其中34例采用注射用甲泼尼龙琥珀酸钠联合静脉用人丙种球蛋白针(A组)，39例采用注射用甲泼尼龙琥珀酸钠联合血液透析进行治疗(B组)；对其一般资料、致病因素、临床特征、实验室检查结果及治疗与转归进行总结。 结果 58例患者有明确病因，其中57例为药物所致，占98.3%；致敏药物以别嘌呤醇和卡马西平为主，分别占34.5%、24.1%。SJS和TEN患者的常见临床特征为体温升高、电解质紊乱、白细胞、肝酶、尿素氮升高，发生率均在30%以上，也有部分伴有血糖升高者，发生率为20.5%；总病原菌检出率91.8%，其中皮肤病原菌检出率最高，占83.6%，菌种主要是金黄色葡萄球菌，占61.6%。A、B两组退热时间、住院时间、死亡率差异无统计学意义(P>0.05)，A组费用较低，差异有统计学意义(P<0.05)。 结论 本地区近2年引起SJS和TEN的常见药物为别嘌呤醇和卡马西平，在对症治疗的基础上，糖皮质激素联合静脉用人丙种球蛋白针或糖皮质激素联合血液透析均能取得较好疗效。      

Toxic epidermal necrolysis in a patient receiving concurrent phenytoin and whole brain and thoracic radiotherapy

Toxic epidermal necrolysis (TEN) is a severe drug induced type IV hypersensitivity syndrome that can be caused by anticonvulsant drugs, especially the aromatic anticonvulsants such as phenytoin. Most patients with brain metastasis receive whole brain radiotherapy along with anti-edema measures and anticonvulsants either as prophylactic or for symptom control; phenytoin being the most commonly used drug. In a subset of patients, cranial irradiation may act as a precipitating factor along with anticonvulsants for the development of TEN. We report a 54-year-old patient with metastatic non-small cell lung cancer treated with palliative whole brain and mediastinal radiotherapy with concurrent phenytoin-developing TEN, which started within the radiation portals with subsequent generalization. Though a rare, but serious complication, avoidance of the use of phenytoin concurrent with radiotherapy, replacing phenytoin with newer anticonvulsants, early recognition, aggressive management and awareness of this possible complication has been implied upon in this report.Steven Johnson Syndrome (SJS), Steven Johnson Syndrome-toxic epidermal necrolysis (SJS-TEN), and toxic epidermal necrolysis (TEN) are a spectrum of type IV hypersensitivity drug-induced disorders, which are characterized by blisters and epidermal detachment resulting from epidermal necrosis in the absence of substantial dermal inflammation often associated with anticonvulsant medications.1,2 Patients with brain metastasis, irrespective of the primary tumor location are often symptomatic. They require active treatment in the form of palliative radiotherapy to the whole brain and symptomatic management, which includes anti-edema measures with systemic steroids and use of anticonvulsants either for seizure control or prophylactic use. Phenytoin is the most common drug used for seizure control. A subgroup of patients receiving this combination of whole brain radiotherapy and phenytoin develop severe cutaneous hypersensitive drug reaction that may manifest as SJS-TEN spectrum of systemic syndrome.3,4 In this report, we describe one case of TEN developing in a patient with metastatic lung cancer receiving palliative whole brain and thoracic radiotherapy (RT) with concurrent phenytoin, emphasizing the need for anticipation, early recognition, and aggressive management of this potentially fatal medical emergency.     

中毒性表皮坏死松解症26例临床分析

目的探讨中毒性表皮坏死松解症（TEN）的临床特征、致敏药物、治疗方法及预后。方法回顾性分析26例TEN患者的临床资料。结果患者平均年龄（44.46±21.87）岁,均伴有黏膜损害,致敏药物以抗生素类、抗癫痫药、解热镇痛药、别嘌呤醇为主,26例均使用大剂量糖皮质激素,7例联合丙种球蛋白治疗。治愈21例,好转3例,死亡2例。结论 TEN是一种累及全身皮肤黏膜严重威胁生命的疾病,病死率高,早期足量使用大剂量激素联合丙种球蛋白治疗为主要的治疗手段,伴有内脏损害和严重感染的患者预后较差。     

弹性髓内钉与加压钢板治疗儿童前臂不稳定骨折比较研究

[目的]比较弹性髓内钉与加压钢板治疗儿童前臂不稳定骨折的疗效。[方法]用弹性髓内钉与加压钢板分别治疗儿童前臂不稳定骨折39例和58例,比较两组病人的手术时间、住院时间、骨折X线愈合时间、术后并发症、经济费用以及前臂骨折治疗效果评价分级（Anderson评价标准）进行功能评分。[结果]所有患儿骨折都临床愈合,两种方法手术时间、住院日、X线愈合时间、总的医疗费用比较比较,差异有统计学意义。[结论]弹性髓内钉治疗儿童前臂不稳定骨折的疗效要优于加压钢板,是一种微创,疗效良好的手术方法。