Toxic Epidermal Necrolysis (Lyell syndrome): a pathology for burn units.

Abstract

Toxic Epidermal Necrolysis (Lyell's syndrome) is a rare but very serious dermatological lesion, characterized by the sudden onset of high fever, signs of systemic toxicity and intense mucocutaneous exfoliation. Its pathophysiology is not yet well determined, although it is almost consensual the presence of an immunological basis. It appears usually as an answer to the taking of a given drug, and, in spite of being self-limited in the absence of complications, if not well managed it is associated with great morbidity and a high mortality, due, in most cases, to the developing of sepsis. Treatment includes mainly the immediate suspension of the inducing drug and the precocious admission of the patient in a hospital facility with the capacity to provide intensive support care and to minimize the infectious risk, having also the conditions for the execution of surgical debridement and covering of the affected areas, that is to say in Burn Units. There are in study several therapeutical measures designed to lower the morbidity and mortality of this syndrome, namely the use of plasmapheresis; the administration of high doses of N-acetylcysteine; immunosuppression; hyperbaric oxygen, etc. The authors present the treatment protocol in use at the Coimbra Burns Unit, in Portugal, illustrated with a clinical case from that Unit.