A comparison of international treatment guidelines for Stevens‐Johnson Syndrome and Toxic Epidermal Necrolysis

Abstract

Stevens‐Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare and life‐threatening mucocutaneous diseases that occur almost exclusively as a result of adverse drug reactions, although there are rare cases attributed to infection, immunization, or malignancy.1,2 Given the low incidence of these diseases as well as the high level of morbidity and mortality, randomized controlled clinical trials are difficult to perform, making it difficult to establish a “gold‐standard” treatment. To date, there are only five published articles in the literature detailing evidence‐based guidelines for the treatment of SJS and TEN, one of which is specifically tailored to pediatric and young adult patients.3–7 These guidelines have significant overlap in regards to the importance of prompt discontinuation of the offending drug and the need for supportive care, but there are differences in regards to the ideal supportive care measures. Additionally, there is still no clear consensus agreement on the pharmacological treatment of SJS and TEN.4–7 Herein, we aim to compare the international treatment guidelines for management of SJS and TEN as well as promote continued discussion and a multidisciplinary approach to establish consensus recommendations for these mucocutaneous emergencies.

Evaluation of the Factors Influencing Mortality in Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Multicenter Study of 166 Patients

“…Sekula et al's large-scale, multinational study followed 460 patients diagnosed with SJS/TEN for 1 year [32]. The 6-week mortality rate within this cohort was 23% (95% CI [19][20][21][22][23][24][25][26][27], with mortality continuing to increase after the 6th week, reaching an overall mortality of 34% (95% CI 30-39) at the end of 1 year. Various factors were found to influence mortality: age, severity of the reaction, recent malignancy, pre-existing severe kidney or liver disorders, and recent infections.…”

Economic Evaluation of HLA-B*15:02 Genotyping for Asian Australian Patients With Epilepsy

“…Given the paucity of literature on SJS/TEN costs within the Australian context, costs were estimated based on current management guidelines that unanimously recommended management in the intensive care unit or specialized burn care centers . We derived the weighted average length of stay of 20.06 days reported at state-referral centers for SJS/TEN .…”

“…Given the paucity of literature on SJS/TEN costs within the Australian context, costs were estimated based on current management guidelines that unanimously recommended management in the intensive care unit or specialized burn care centers. 47 We derived the weighted average length of stay of 20.06 days reported at state-referral centers for SJS/TEN. 26,27 An estimate of SJS/TEN management costs was obtained by multiplying the length of stay by the average cost per patient bed-day in an Australian intensive care unit.…”

Evolving trends in inpatient paediatric dermatology

“…Aside from the recommendation of immediate medication withdrawal and supportive care, there are no consensus guidelines for the systemic treatment of SJS/TEN, likely due to the challenge of performing randomized controlled trials for a disease with low incidence and high mortality. Recently, Pisano et al [18 ▪ ] compared five international treatment guidelines for the management of SJS/TEN, which included one specific to the paediatric population. All guidelines recommend admission to an intensive care or burn unit, involvement of a multidisciplinary team, an emphasis on supportive care, and institution-specific protocols and order sets [18 ▪ ].…”

“…Recently, Pisano et al [18 ▪ ] compared five international treatment guidelines for the management of SJS/TEN, which included one specific to the paediatric population. All guidelines recommend admission to an intensive care or burn unit, involvement of a multidisciplinary team, an emphasis on supportive care, and institution-specific protocols and order sets [18 ▪ ]. All guidelines unanimously agreed that there are insufficient data to make recommendations for the use of one systemic therapy over the other for pharmacologic treatment.…”