The management of toxic epidermal necrolysis

Abstract

The mortality rate of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is high; approximately 5% for SJS and 25% for TEN. It is therefore vital for the treating physician to recognise SJS and TEN promptly through the identification of these diseases' characteristic clinical features so that the offending drug is promptly withdrawn, supportive therapy is administered and adjunctive therapies are considered. Supportive therapy addressing the manifestations and complications of acute skin failure include monitoring the fluid - electrolyte balance and providing enteral or parenteral nutrition, wound care and treatment of sepsis. In addition, supportive care of the affected mucosal surfaces is required through the use of aggressive ocular lubrication, topical corticosteroids, hygienic mouthwashes and oral anaesthetics, together with monitoring for urinary retention. There is sufficient evidence for the use of intravenous immune globulin in the acute management of SJS/TEN provided an adequate dose of 2-3 g/kg is administered. Cyclosporine appears to also be an effective agent although randomised control studies are required to demonstrate its benefit and establish the dose, duration of therapy and safety profile. The role of corticosteroids is currently under revision. Some earlier studies have shown a lack of efficacy or increased mortality in their use but the use of high doses early in the course of disease may actually reduce morbidity and mortality. The role of plasmapheresis, anti-tumour necrosis factor (TNF) biologics and N-acetylcysteine is promising but further studies are required to elucidate their benefit. Preventative strategies such as pharmacogenetic screening needs to be strongly considered, with the provision of cost-effective assays with a rapid turn-around time.

Patterns and Outcomes of Cutaneous Adverse Drug Reactions in a Hospital Based Study

“…In general, the mortality rate in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) is high; approximately 5% for SJS and 30% for TEN. 29 In one of the studies, 4 out of 7 cases of TEN expired, which comprised 40% of all ADR related mortali es. 20 In another study, 5 out of 12 cases of TEN and 2 out of 3 cases of erythroderma succumbed.…”

Síndrome de Stevens-Johnson (SSJ) em adultos: revisão sistemática.

“…2017 Stevens e também pelo Dr. Frank Chambliss Johnson, pediatras estadunidenses que publicaram conjuntamente uma descrição dessa desordem no American Jornal of Diseases (AJD), enquanto uma síndrome mucocutânea aguda em dois (02) rapazes (NGAN;OAKLEY, 2014;SO-TELO-CRUZ, 2012;FERNANDO, 2012;ARGUIJO et al, 2010;HARR;FRENCH, 2010;GUTIÉRREZ;REPETTO, 2004;CROSS;BORGES;ESTIVEZ 2004;LONG et al, 1992).…”

A Review of the Active Treatments for Toxic Epidermal Necrolysis

“…Although there have been no randomized controlled trials done, cyclosporine tends to be beneficial for the treatment of TEN (Table 3) 24 27 . The doses (3 10 mg/kg/day) and durations vary but have been reported to arrest the disease progression and promote re-epithelialization 9,12 . A limitation in the active use for the treatment of TEN is that the full therapeutic effect takes more than 1 month.…”