Roujeau Jean-Claude
Centre de référence maladies rares, dermatoses bulleuses immunologiques et toxiques, service de dermatologie, hôpital Henri-Mondor, Inserm U659, Université Paris-XII, 94010 Créteil.
Rev Prat. 2007 Jun 15;57(11):1165-70.
Epidermal necrolysis (Stevens-Johnson syndrome, toxic epidermal necrolysis) is an acute and severe skin disease, induced by "(drug allergy" and characterized by the destruction of the epithelium of the skin and mucous membranes. It is extremely rare: about 2 cases per million per year. It is a life-threatening emergency. Blisters and detachment may involve a high portion of the body surface area and several mucosal sites. Visceral complications are frequent. The clinical diagnosis should be confirmed by a skin biopsy showing full-thickness necrosis of the epidermis. A dozen "high risk" medications account for 50% of cases. Symptomatic management in specialized units is urgent. The mortality rate is high (20-25%) and about one half of survivors will have sequelae, especially on the eyes.
表皮坏死松解症(史蒂文斯-约翰逊综合征、中毒性表皮坏死松解症)是一种急性重症皮肤病,由“药物过敏”诱发,其特征为皮肤和黏膜上皮遭到破坏。该病极为罕见:每年每百万人中约有2例。这是一种危及生命的急症。水疱和皮肤脱落可能累及身体大部分表面积以及多个黏膜部位。内脏并发症很常见。临床诊断需通过皮肤活检证实表皮全层坏死来确诊。十几种“高风险”药物导致了50%的病例。在专科病房进行对症治疗刻不容缓。死亡率很高(20%-25%),约一半幸存者会有后遗症,尤其是眼部。