Background:
Toxic epidermal necrolysis (TEN) is a severe immune mediated muco-cutaneous drug reaction. TEN is a cytotoxic T-lymphocyte mediated process involving desquamation and blistering of skin and mucosa over a large body surface area. Stevens-Johnson Syndrome (SJS) and TEN are considered a spectrum of the same disease process with <10%, and >30% BSA affected respectively. Although rare, TEN is associated with a mortality rate of 30% and significant long-term morbidity for survivors. Standardised protocols for management of TEN and SJS remain a point of contention.
Methods:
A literature review on the immediate management of TEN and SJS was conducted. Studies concerned with mortality rates of TEN and SJS and therapeutic efficacy were included in the analysis including review articles, RCT, and retrospective studies; representing significant heterogeneity within the literature.
Results:
Multiple different immunomodulatory treatments have been proposed in recent literature. A recent Meta-analysis by Tsai et al (2020) demonstrated that corticosteroid and IV Immunoglobulin infusion was the only systemic therapy to show survival benefit over supportive care alone.
Discussion:
Supportive care in combination with immune modulating therapy represents the best survival outcomes for patients with TEN and SJS. The authors recommend the following key features in the treatment of TEN:
- Consider the diagnosis early to enable timely recognition and cessation of the precipitant.
- Treatment should be provided in a specialised major burns centre, as per ANZBA major burns criteria. Early consultation of dermatology, plastic surgery and ophthalmology services are crucial.
- Surgical debridement not recommended in the majority of cases.
- Adequate nutritional support, preferably via enteral route.
- Combination corticosteroid and IV Immunoglobulin infusion therapy is shown to improve mortality in recent literature.
Conclusion:
Review of recent literature suggests early diagnosis, specialised supportive care, and combination corticosteroid and IV Immunoglobulin therapy may improve survival in TEN/SJS patients.