Roujeau J C, Chosidow O, Saiag P, Guillaume J C
Department of Dermatology, Hôpital Henri Mondor, Université Paris XII.
J Am Acad Dermatol. 1990 Dec;23(6 Pt 1):1039-58. doi: 10.1016/0190-9622(90)70333-d.
Toxic epidermal necrolysis is perhaps the most formidable disease encountered by dermatologists. Uncommon but not rare, toxic epidermal necrolysis occurs in 60 to 70 persons per year in France. It remains as puzzling a disorder as it was 34 years ago, when described by Lyell. Whether or not toxic epidermal necrolysis is the most severe form of erythema multiforme is still the subject of discussion. The physiopathologic events that lead to this rapidly extensive necrosis of the epidermis are not understood. Indirect evidence suggests a hypersensitivity reaction, but the search for potential immunologic mechanisms has resulted in little data to support this hypothesis. Accumulated clinical evidence points to drugs as the most important, if not the only, cause of toxic epidermal necrolysis. Sulfonamides, especially long-acting forms, anticonvulsants, nonsteroidal anti-inflammatory agents, and certain antibiotics are associated with most cases of toxic epidermal necrolysis. Many other drugs have been implicated in isolated case reports. All organs may be involved either by the same process of destruction of the epithelium as observed in the epidermis or by the same systemic consequences of "acute skin failure" as seen in patients with widespread burns. Sepsis is the most important complication and cause of death. Approximately 20% to 30% of all patients with toxic epidermal necrolysis die. Elderly patients and patients with extensive lesions have a higher mortality rate. Surviving patients completely heal in 3 to 4 weeks, but up to 50% will have residual, potentially disabling ocular lesions. The prognosis is improved by adequate therapy, as provided in burn units, that is, aggressive fluid replacement, nutritional support, and a coherent antibacterial policy. Corticosteroids, advocated by some in high doses to halt the "hypersensitivity" process, have been shown in several studies to be detrimental and should be avoided.
中毒性表皮坏死松解症或许是皮肤科医生所遇到的最可怕的疾病。中毒性表皮坏死松解症并不常见但也并非罕见，在法国每年有60至70人发病。它依然像34年前莱尔所描述时那样，是一种令人困惑的病症。中毒性表皮坏死松解症是否为多形红斑最严重的形式仍是讨论的话题。导致表皮迅速广泛坏死的生理病理过程尚不清楚。间接证据提示为超敏反应，但对潜在免疫机制的探寻几乎没有得到支持这一假说的数据。累积的临床证据表明，药物即便不是中毒性表皮坏死松解症唯一的重要病因，也是最重要的病因。磺胺类药物，尤其是长效制剂、抗惊厥药、非甾体抗炎药以及某些抗生素与大多数中毒性表皮坏死松解症病例相关。许多其他药物在个别病例报告中也有牵连。所有器官都可能受累，要么是通过与表皮所见相同的上皮破坏过程，要么是通过与大面积烧伤患者所见相同的“急性皮肤衰竭”的全身后果。脓毒症是最重要的并发症和死亡原因。所有中毒性表皮坏死松解症患者中约20%至30%死亡。老年患者和皮损广泛的患者死亡率更高。存活患者在3至4周内完全愈合，但高达50%的患者会有残留的、可能导致残疾的眼部病变。如烧伤病房所提供的充分治疗，即积极的液体复苏、营养支持和连贯的抗菌策略，可改善预后。一些人主张大剂量使用皮质类固醇来阻止“超敏反应”过程，但多项研究表明其有害，应避免使用。
