From the Guidelines
Toxic epidermal necrolysis (TEN) requires immediate hospitalization in a burn unit or intensive care unit, and treatment involves stopping the suspected triggering medication and providing supportive care, including fluid and electrolyte management, wound care, pain control, and nutritional support. The condition is a severe, life-threatening skin reaction characterized by widespread death of skin cells, causing the epidermis to separate from the dermis. According to the U.K. guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults 2016 1, if SJS/TEN is suspected, discontinue any potential culprit drug immediately, and the patient should undergo an evaluation of the critical disease components.
Key Considerations
- Immediate discontinuation of the suspected triggering medication is crucial 1
- Supportive care, including fluid and electrolyte management, wound care, pain control, and nutritional support, is essential
- Intravenous immunoglobulin (IVIG) at 2-3 g/kg total dose over 3-5 days may be used to halt disease progression, although its effectiveness is still being studied 1
- Cyclosporine at 3-5 mg/kg/day for 7-10 days has shown benefit in some cases
- Systemic corticosteroids remain controversial but may be considered in early stages 1
- Meticulous wound care using non-adherent dressings and antiseptic solutions is crucial to prevent infection
Ocular Complications
TEN can also cause severe ocular complications, including conjunctivitis, corneal ulceration, and vision loss 1. Early intervention is critical to prevent late ocular complications, and medical management with topical lubricants, antibiotics, and anti-inflammatories is recommended. Amniotic membrane transplantation may also be useful in stabilizing the ocular surface and preventing cicatrization and conjunctival and corneal defects.
Mortality and Morbidity
TEN has a mortality rate of 25-30%, with sepsis being the leading cause of death 1. Survivors should receive counseling about avoiding the triggering medication and potentially cross-reactive drugs in the future. The condition results from an immune-mediated reaction, typically triggered by medications such as anticonvulsants, antibiotics (particularly sulfonamides), allopurinol, and certain NSAIDs.
From the Research
Definition and Causes of Toxic Epidermal Necrolysis
- Toxic epidermal necrolysis (TEN) is a severe adverse drug reaction associated with the separation of skin and mucous membranes at the dermal-epidermal junction 2.
- Almost all cases of TEN appear to be caused by an idiosyncratic drug reaction 3.
- The disorder initially presents with fever, malaise, and painful mucosal ulcerations, but progresses to widespread cutaneous lesions 4.
Treatment Options for Toxic Epidermal Necrolysis
- Active interventions performed to date include the use of systemic corticosteroids, intravenous immunoglobulins (IVIg), cyclosporine, plasmapheresis, anti-tumor necrosis factor drugs, and N-acetylcysteine, but none has been established as the most effective therapy 2.
- IVIg and short-term high-dose corticosteroids were regarded as the most promising treatments for TEN in a comprehensive review of all reported TEN cases from 1975-2003 2.
- Cyclosporine has been shown to improve survival in TEN patients compared to supportive care and intravenous immunoglobulin 5.
- Infliximab seems to be effective in the treatment of TEN, especially in cases of rapid progression 6.
Management and Care of Patients with Toxic Epidermal Necrolysis
- Nursing on a specialized unit is of paramount importance 3.
- Patients with TEN are difficult to handle in intensive-medical care, whereby attention should especially be paid to sufficient pain therapy, and the positioning of the patient is a particular challenge 6.
- A multifaceted regimen appears indicated, targeting various likely intermediary mechanisms, including elimination of residual drug, immunosuppression, inhibition of death receptor pathways, general antiapoptotic strategies, and aggressive supportive care 3.