What is the management of Toxic Epidermal Necrolysis (TEN)?

From the Guidelines

Management of toxic epidermal necrolysis (TEN) requires immediate hospitalization in a burn unit or intensive care setting, with a focus on supportive care, including fluid and electrolyte management, temperature regulation, nutritional support, and meticulous wound care. The first step is to identify and discontinue the causative agent, typically a medication like antibiotics, anticonvulsants, or NSAIDs. Supportive care is crucial and includes:

• Fluid and electrolyte management, with fluid replacement volumes determined by the formula: body weight/% BSA epidermal detachment, as suggested by Shiga and Cartotto 1
• Temperature regulation
• Nutritional support, often 1.5-2 times basal energy requirements
• Meticulous wound care with non-adherent dressings changed daily, and the use of an appropriate dressing on exposed dermis to reduce fluid and protein loss, limit microbial colonization, and help pain control 1 Pain management with opioid analgesics is essential. Infection prevention involves regular cultures of wounds, blood, and urine, with antibiotics only used for confirmed infections, not prophylactically. For specific treatments, high-dose intravenous immunoglobulin (IVIG) at 1-3 g/kg/day for 3-5 days may be beneficial if started early. Systemic corticosteroids remain controversial but may be used in early stages (methylprednisolone 1-2 mg/kg/day for 3-5 days), as some studies suggest a lower mortality with their use 1. Cyclosporine (3-5 mg/kg/day) has shown promise in reducing mortality and disease progression. TNF-alpha inhibitors like etanercept (25-50 mg twice weekly) or infliximab (single dose of 5 mg/kg) may be considered in severe cases. TEN has a high mortality rate (25-35%) due to sepsis, multiorgan failure, and fluid/electrolyte imbalances, so aggressive management in specialized centers is essential for improving outcomes. The skin management regimen should include careful handling of the skin to minimize shearing forces, and the use of antishear handling, as well as day-to-day bedside care by specialist nurses familiar with skin fragility disorders 1. A surgical approach, involving debridement of detached epidermis and physiological wound closure using biosynthetic xenograft or allograft, may be considered in cases where conservative management fails 1.

From the Research

Management of Toxic Epidermal Necrolysis

• The management of toxic epidermal necrolysis (TEN) involves immediate withdrawal of the causative drug and early transfer to a burn centre for specific and intensive care 2.
• The mainstay of treatment for TEN includes discontinuation of the offending drug, specialized care in an intensive care unit or burn center, and supportive therapy 3.
• SCORTEN is a scoring system used to stratify severity and predict mortality, and it seems to be an accurate scoring system for estimation of mortality rate 2, 4.
• Pharmacogenetic studies have clearly established a link between human leukocyte antigen allotype and TEN, and human leukocyte antigen testing should be performed on patients of East Asian descent before the initiation of carbamezapine and on all patients before the initiation of abacavir 3.
• The effectiveness of systemic steroids, intravenous immunoglobulins, plasmapheresis, cyclosporine, biologics, and other agents is uncertain 3, but some studies suggest that the administration of short-term dexamethasone pulse therapy may be beneficial in reducing the mortality rate 5.
• IVIg did not significantly alter mortality in some studies 4.

Treatment Options

• Supportive care, intravenous immunoglobulin, and cyclosporine are some of the treatment options used for TEN 4.
• Dexamethasone pulse therapy has been used in some cases, particularly during the initial phase, and may be beneficial in reducing the mortality rate 5.
• Immunomodulating systemic therapy is also considered in the treatment of TEN 6.

Prognosis and Mortality

• TEN has a high mortality rate, making early diagnosis and treatment of paramount importance 3.
• The overall observed mortality rate was 47.6% in one study, and the mortality rate predicted by SCORTEN was 42.2% 2.
• Age older than 40 years, the presence of metabolic syndrome and/or gout, higher body surface area involvement, higher SCORTEN, and higher number of medical comorbidities statistically significantly increased risk of death 4.