[Toxic epidermal necrolysis and Stevens-Johnson syndrome].
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.
Roujeau
Jean-Claude
JC
Centre de référence maladies rares, dermatoses bulleuses immunologiques et toxiques, service de dermatologie, hôpital Henri-Mondor, Inserm U659, Université Paris-XII, 94010 Créteil. jean-claude.roujeau@hmm.aphp.fr
D000328:Adult; D001706:Biopsy; D003937:Diagnosis, Differential; D005602:France / Q000453:epidemiology; D005817:Genetic Counseling; D006801:Humans; D011379:Prognosis; D012307:Risk Factors; D012867:Skin / Q000473:pathology; D013262:Stevens-Johnson Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy