Stevens-Johnson 综合征和中毒性表皮坏死松解症

表现为发热，皮疹，淋巴结肿大，关节痛，肝炎，肾炎，心肌炎，嗜酸粒细胞增多和异型淋巴细胞。[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[57]Husain Z, Reddy BY, Schwartz RA. DRESS syndrome: Part I. Clinical perspectives. J Am Acad Dermatol. 2013;68:693.http://www.ncbi.nlm.nih.gov/pubmed/23602182?tool=bestpractice.com[58]Husain Z, Reddy BY, Schwartz RA. DRESS syndrome: Part II. Management and therapeutics. J Am Acad Dermatol. 2013;68:709.http://www.ncbi.nlm.nih.gov/pubmed/23602183?tool=bestpractice.com

最常见的致病药物是苯妥英、苯巴比妥和卡马西平以及磺胺类药物。[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[59]Baba M, Karakas M, Aksunger VL, et al. The anticonvulsant hypersensitivity syndrome. J Eur Acad Dermatol Venereol. 2003;17:399-401.http://www.ncbi.nlm.nih.gov/pubmed/12834448?tool=bestpractice.com

更常见于黑人。

更多见于黑人。 从开始用药到出现皮疹，需要2~6周。[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

皮肤活检和临床表现;FBC可能会出现嗜酸性粒细胞增多;如果有肝炎肝功能检测肝酶可升高。

婴儿期或儿童患病（Ritter病）。

细菌内毒素破坏表皮，但没有坏死或炎症。[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

FBC，血或创面的菌培养，和皮肤活检。

二者中任一 葡萄球菌 或 链球菌 均可成为感染源，表现为脓毒血症的体征和晒伤样皮疹。

FBC，血或创面的菌培养，和皮肤活检。

患者出现皮疹，但在移植物抗宿主病的皮肤没有Stevens-Johnson综合征（SJS）和中毒性表皮坏死松解症（TEN）那样容易剥脱。

接受移植的患者出现皮疹是因为排异，而不是药物引起的反应，但 IV 级皮肤表现可能像 TEN 中一样严重。 眼部受累罕见。[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

FBC和皮肤活检。

应用光气肟或路易氏剂会立即产生临床可见的皮疹，如红斑和水疱，而应用芥子气则临床表现会推迟2-24小时出现。[60]Centers for Disease Control and Prevention. Toxic syndrome description: vesicant/blister agent poisoning. April 2013. http://www.emergency.cdc.gov (last accessed 23 September 2017).http://emergency.cdc.gov/agent/vesicants/tsd.asp

皮肤活组织检查。

靶型损害通常由疱疹病毒引起。[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

皮肤活组织检查。

创伤的结果造成不同程度表皮及真皮的破坏。[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

如果没有明确的病史可做皮肤活检。

疱病合并恶性肿瘤。[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

皮肤活检和直接免疫荧光。

再次应用致敏药物皮疹出现在相同部位;很少出现系统症状;圆形红斑或有水疱的斑片。[61]Sehgal V, Srivastava G. Fixed drug eruption (FDE): changing scenario of incriminating drugs. Int J Dermatol. 2006; 45:897-908.http://www.ncbi.nlm.nih.gov/pubmed/16911371?tool=bestpractice.com[62]Lipowicz S, Sekula P, Ingen-Housz-Oro S, et al. Prognosis of generalized bullous fixed drug eruption: comparison with Stevens-Johnson syndrome and toxic epidermal necrolysis. Br J Dermatol. 2013; 168:726-732.http://www.ncbi.nlm.nih.gov/pubmed/23413807?tool=bestpractice.com

皮肤活组织检查。

发热、中性粒细胞增多、急性无菌脓疱、红斑、面部水肿。[63]Thienvibul C, Vachiramon V, Chanprapaph K. Five-year retrospective review of acute generalized exanthematous pustulosis. Dermatol Res Pract. 2015;2015:260928.http://www.hindawi.com/journals/drp/2015/260928/http://www.ncbi.nlm.nih.gov/pubmed/26783390?tool=bestpractice.com

皮肤活组织检查。

未摄入药物；发热，手、舌、臀部的丘疱疹以及口腔溃疡。[64]Chung WH, Shih SR, Chang CF, et al. Clinicopathologic analysis of coxsackievirus A6 new variant induced widespread mucocutaneous bullous reactions mimicking severe cutaneous adverse reactions. J Infect Dis. 2013;208:1968-1978.http://jid.oxfordjournals.org/content/208/12/1968.longhttp://www.ncbi.nlm.nih.gov/pubmed/23904296?tool=bestpractice.com

皮肤活检；皮肤和水疱液中有病毒。