Stevens-Johnson综合征(SJS)和中毒性表皮坏死松解症(TEN)可能与之前使用药物相关,最多见的是抗惊厥药、抗生素和非甾体类抗炎药。 其他因素包括感染。
阅读更多患者可表现为尼氏征阳性,即对水疱或红斑施压后表皮很容易脱落。
根据临床表现可诊断,皮肤组织病理检查可确诊。
一旦诊断致敏药物应该停止,治疗只是支持性的。
若确诊或怀疑此诊断最好尽快将患者送至烧伤中心护理创面。
大多数SJS患者痊愈(死亡率1%至5%)。 但再次应用相同或其他药物可复发。
TEN患者死亡率更高
从长远来看,患者应确保不再接触致敏药物,自行用药应谨慎。 皮肤愈合过程中应防晒和润肤。
目前,如果有风险的患者即将接受如卡马西平、别嘌呤醇或甲氧苄啶/磺胺甲噁唑等药物治疗,可能需要 HLA 筛查。
Stevens-Johnson 综合症 (SJS) 是一种严重的皮肤剥脱,伴有黏膜皮肤并发症。[1]Stevens AM, Johnson FC. A new eruptive fever associated with stomatitis and ophthalmia. Am J Dis Child. 1922;24:526-533. Stevens-Johnson综合征(SJS)是一种严重的累及皮肤黏膜的表片剥脱性皮炎 是对外来抗原的免疫反应。[2]Dodiuk-Gad RP, Chung WH, Valeyrie-Allanore L, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis: an update. Am J Clin Dermatol. 2015;16:475-493.http://www.ncbi.nlm.nih.gov/pubmed/26481651?tool=bestpractice.com SJS比重症多形红斑更严重,轻于TEN,其分类是根据皮肤受累的面积,SJS:小于10%体表面积(TBSA)受累;SJS/TEN重叠:10%~30%TBSA受累;TEN:大于30%TBSA。[3]Mockenhaupt M. Stevens-Johnson syndrome and toxic epidermal necrolysis: clinical patterns, diagnostic considerations, etiology, and therapeutic management. Semin Cutan Med Surg. 2014;33:10-16.http://www.ncbi.nlm.nih.gov/pubmed/25037254?tool=bestpractice.com[4]Roujeau JC, Stern RS. Severe adverse cutaneous reactions to drugs. N Engl J Med. 1994 Nov 10;331(19):1272-85.http://www.ncbi.nlm.nih.gov/pubmed/7794310?tool=bestpractice.com[5]Bastuji-Garin S, Rzany B, Stern RS, et al. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol. 1993 Jan;129(1):92-6.http://www.ncbi.nlm.nih.gov/pubmed/8420497?tool=bestpractice.com[6]Auquier-Dunant A, Mockenhaupt M, Maldi L, et al. Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study. Arch Dermatol. 2002;138:1019-1024.http://www.ncbi.nlm.nih.gov/pubmed/12164739?tool=bestpractice.com[7]Lyell A. Toxic epidermal necrolysis: an eruption resembling scalding of the skin. Br J Dermatol. 1956;68:355-361.http://www.ncbi.nlm.nih.gov/pubmed/13374196?tool=bestpractice.com[8]Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis. Part I: introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol. 2013;69:173.http://www.ncbi.nlm.nih.gov/pubmed/23866878?tool=bestpractice.com[9]Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis. Part II: prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol. 2013;69:187.http://www.ncbi.nlm.nih.gov/pubmed/23866879?tool=bestpractice.com