史蒂芬斯-强森症候群

title: 由来.
content: 史提芬强生症候群是由于两位美国的儿科医生,Albert Mason Stevens 和 Frank Chambliss Johnson,两人于1922年在《American Journal of Diseases of Children》联合发表了一份关于此疾患的描述,因此而被命名。
title: 流行病学.
content: 史提芬强生症候群是一种罕见疾病,每年大约有1,000,000分之2.6至6.1个个案。在美国,每年大约有300个新症。比较儿童,情况较多发生于成人,而女性较男性普遍,两性的比例为3:2。
title: 分类.
content: 在医学文献中有个协议指史提芬强生症候群 (SJS)可以被认为是中毒性表皮坏死症(Toxic epidermal necrolysis/TEN)的一个较温和的形式。这个情况在1992年首次得到承认。  两种疾病可以弄错为多型红斑。 多型红斑有时是由药物反应造成,但较常的是过敏症反应第四型 (最普遍由单纯疱疹引致) 并且相对地是良性的。虽然SJS和TEN都能由感染造成,他们最常都是药物的不良反应。他们潜在的后果比多型红斑更危险。
title: 症状.
content: 史蒂芬斯-强森症候群的特征是会先出现类似流行性感冒的前驱症状,包括发烧、喉咙痛、头痛,接着在皮肤出现钱币大小的环状纹(标靶病征),面积逐渐扩大,随后出现水疱。患病部位若超过体表面积百分之卅以上,则称为毒性表皮坏死症候群或称莱尔症状群(Lyell syndrome)。
title: 成因.
title: 感染.
content: 它可以由感染造成,通常是以下的感染,例如:单纯性泡疹病毒(herpes simplex virus)、流行性感冒(influenza)、腮腺炎(mumps)、猫爪热(cat-scratch fever)、组织胞浆菌病(histoplasmosis)、人类疱疹病毒第四型(HHV-4)(Epstein-Barr virus)、肺炎霉浆菌(mycoplasma pneumoniae)或类似感染。
title: 药物.
content: 因为药物过敏 (例如盘尼西林、巴比妥盐、磺胺剂、普拿疼、抗癫痫药物等等。) 或因为癌症等原因所致,但也有部分是不明原因的。此外亦有病例报告过,一些草药过敏如人参,或毒品如古柯碱。
title: 遗传.
content: 在一些东亚人口研究 (汉人与泰人),卡马西平(Carbamazepine)和苯妥英钠(Phenytoin)与HLA-B*1502 (HLA-B75)会导致史提芬强生症候群有强烈的反应,HLA-B serotype 较宽阔serotype HLA-B15。一项在欧洲的研究建议指出指标基因(Genetic marker)只是与亚洲东部的人有关。根据亚洲人的发现,在欧洲进行相似的研究显示61%的黄嘌呤诱导史提芬强生症候群/中毒性表皮坏死病患者带有HLA-B58 (B*5801 对偶基因 - 欧洲人的显性频率具代表性地有3%)。一项研究推断"即使当HLA-B 等位基因表现是高风险因素,至于别嘌醇,它们既不充分也不必要的去解释疾病。"
title: 治疗.
content: 治疗第一步是要停止造成它过敏的药物。其处置的原则和烧/烫伤的治疗类似,包含给予静脉水分补充、症状治疗(如止痛等)。迄今它并无特效药可以处理,类固醇的使用目前尚有争议,而且可能造成次发性的感染。近年有提出利用化学药物治疗的建议,但尚未达成共识。此外如果眼部也有发病时,应协寻眼科医生会诊。
title: 愈后.
content: 史蒂芬斯-强森症候群是约有百分之五的致命率。目前以史可尔登指数(SCORTEN scale)可以约略估计预后死亡率。其他的并发症包括多重器官衰竭、角膜脱落及失明。

Summary: 史蒂芬斯-强森综合征(英语:Stevens-Johnson syndrome,缩写为 SJS),又称史提芬强生综合征、史帝文生氏-强生综合征、史帝文生-强生综合征,是"多型性红斑"(Erythema multiforme)的一种,能影响表皮的细胞死亡,导致真皮与表皮分离,是一种可致命的皮肤疾病。被认为是一种皮肤与黏膜严重过敏反应。亦被医学界认定为是多型性红斑的严重型。虽然多数的个案都是原发的,已知的起因主要是药物治疗而引起的,再则是受到病菌感染及癌症(很少情况)所触发的。目前多将由药物造成的症状的称为史蒂芬斯-强森综合征,由病毒产生的症状则称为多型性红斑。

Stevens–Johnson syndrome: Stevens–Johnson syndrome (SJS) is a type of severe skin reaction. Together with toxic epidermal necrolysis (TEN) and Stevens–Johnson/toxic epidermal necrolysis (SJS/TEN), it forms a spectrum of disease, with SJS being less severe. Erythema multiforme (EM) is generally considered a separate condition. Early symptoms of SJS 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. The most common cause is certain medications such as lamotrigine, carbamazepine, allopurinol, sulfonamide antibiotics and nevirapine. Other causes can include infections such as "Mycoplasma pneumoniae" and cytomegalovirus, or the cause may remain unknown. Risk factors include HIV/AIDS and systemic lupus erythematosus. The diagnosis of Stevens–Johnson syndrome is based on involvement of less than 10% of the skin. It is known as TEN when more than 30% of the skin is involved and considered an intermediate form when 10–30% is involved. SJS/TEN reactions are believed to follow a type IV hypersensitivity mechanism. It is also included with drug reaction with eosinophilia and systemic symptoms (DRESS syndrome), acute generalized exanthematous pustulosis (AGEP) and toxic epidermal necrolysis in a group of conditions known severe cutaneous adverse reactions (SCARs). Treatment typically takes place in hospital such as in a burn unit or intensive care unit. Efforts may include stopping the cause, pain medication, antihistamines, antibiotics, intravenous immunoglobulins or corticosteroids. Together with TEN, SJS affects 1 to 2 people per million per year. Typical onset is under the age of 30. Skin usually regrows over two to three weeks; however, complete recovery can take months. Overall, the risk of death with SJS is 5 to 10%.