Vnitr Lek 2016, 62(1):62-69

Toxic epidermal necrolysis

Dina Odarčenková^*, Milan Kvapil

Interní klinika 2. LF UK a FN Motol Praha

Toxic epidermal necrolysis is a rare, acute and life-threatening disorder manifested by extensive separation of the epidermis and mucosal surfaces. It is a serious adverse response to administered drugs, mostly antibiotics, anticonvulsants or NSAIDs. First described by Scottish dermatologist Alan Lyell in 1956, it is also known as Lyell's syndrome. Mortality of the disease is high, therefore its early diagnosis is crucial and immediate initiation of appropriate patient care necessary. It is a rare disease with a very low incidence and serious prognosis, which is a considerable hindrance to undertaking large randomized clinical studies. It is therefore difficult to evaluate the effectiveness of various therapeutic options. As the most promising so far appears the administration of intravenous immunoglobulins, apparently reaching improvement in the clinical condition of the patient, with a good tolerances and minimal side adverse effects. New experimental techniques endeavour to seek a way to the therapy through targeted influencing of granulysin as the major cytotoxic mediator.

Keywords: adverse drug reaction; corticosteroids; cyclosporine A; granulysin; intravenous immunoglobulins; interdisciplinary team care; keratinocyte apoptosis; medical history; Nikolsky's sign; plasmapheresis; SCORTEN score; Stevens-Johnson syndrome; toxic epidermal necrolysis

Received: May 19, 2015; Accepted: September 29, 2015; Published: January 1, 2016  Show citation