title: Toxic epidermal necrolysis.
abstract: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), also known as Lyell's syndrome, are rare, life- -threatening diseases that are characterised by extensive epidermal detachment, erosion of mucous membranes and severe systemic symptoms. In the majority of cases, the development of symptoms can be attributed to the use of drugs; therefore, the disease pathology is thought to be caused by a severe adverse reaction to drugs. The high mortality rate results primarily from the development of complications in the form of systemic infections and multiple organ failure. TEN and SJS affect all age groups, including newborns, infants and older children. The rarity of these syndromes has not permitted large, randomised studies, which has resulted in numerous difficulties in their diagnosis and management. Because the pathogenesis has not yet been established, the management and systemic treatment of these syndromes have not been standardised. The efficacy of the treatment options suggested has not been confirmed by clinical studies involving suitably large groups of patients, especially children.
authors.affiliation: Department of Paediatric Anaesthesiology and Intensive Therapy, COPERNICUS LLC, Gdańsk, Poland. johika@wp.pl.
authors.forename: Joanna
authors.initials: J
authors.lastname: Hinc-Kasprzyk
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Polak-Krzemińska
authors.forename: Irena
authors.initials: I
authors.lastname: Ożóg-Zabolska
mesh_terms: D002648:Child; D002675:Child, Preschool; D064420:Drug-Related Side Effects and Adverse Reactions / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D009102:Multiple Organ Failure / Q000209:etiology*; D013262:Stevens-Johnson Syndrome / Q000150:complications / Q000503:physiopathology* / Q000628:therapy