Overview

Toxic epidermal necrolysis (TEN) or Lyell’s syndrome is a rare but severe skin condition that can be life-threatening. It causes large areas of skin to peel off, exposing raw, sensitive layers of skin. This condition often occurs as a reaction to medications like antibiotics, anti-inflammatory painkillers or anticonvulsants. Due to the significant risk of infection and fluid loss, effective wound care and infection prevention are essential to saving lives.

What is TEN?

TEN was first described in 1956 by Alan Lyell as “an eruption resembling scalding of the skin."¹ TEN is the most severe form of a group of conditions known as severe cutaneous adverse reactions (SCARs) that also includes Stevens-Johnson Syndrome (SJS). Both result from an intense immune reaction that attacks the skin. In TEN, the outer skin layer (epidermis) separates from the inner layer, exposing the sensitive dermis. This damage can cover more than 30% of the body—making it a medical emergency. Up to 46% of people with TEN will die within six weeks of the onset of symptoms, with survivors facing long-term, life-altering outcomes.²

Individuals with TEN may initially experience fever and body aches that resemble flu-like symptoms. Red, painful skin patches then develop and quickly worsen. These patches can form blisters and peel away, leaving areas that resemble severe burns. The loss of skin causes fluid and electrolyte loss from the damaged areas and an increased risk of infection. The condition can spread to the mouth/throat, eyes or genitals/urethra/anus. Complications include sepsis, dehydration, pneumonia and multiple organ failure. 

Causes of TEN 

TEN is rare.³ Drugs are the primary cause of TEN, but infection (particularly Mycoplasma pneumoniae) has been known to cause the condition.⁴ Up to 15 to 30% of TEN cases have no identifiable cause. ⁴ What triggers TEN is not definitively known, but it is thought to be caused by an immune response following T-cell activation and granulysin-mediated necrosis (cell death).⁴  

Drugs thought to be responsible for TEN include: 

• Allopurinol
• Antibiotics (e.g. sulfonamides)
• Antiepileptic medications (lamotrigine, carbamazepine, phenytoin, phenobarbital)
• Oxicam non-steroidal anti-inflammatory drugs (e.g. meloxicam and piroxicam)
• Nevirapine (used in combination with other drugs to treat human immunodeficiency virus/HIV) 
• Sulfasalazine 

In children, infections such as cold sores, the common cold, influenza and glandular fever can cause TEN. 

Risk factors for developing TEN include:

• A history of TEN 
• A weakened immune system (e.g. from  HIV, autoimmune disease or following chemotherapy)
• A close family member has had TEN (genetic inheritance)

Wound care

Recovery from TEN  can take several weeks or months depending on severity. Wound care for TEN is comparable to burn treatment. Initial management of TEN involves:

• Stopping the drug suspected of causing the reaction 
• Immediate referral to a burn unit or intensive care unit with experience in treating similar cases⁵ 

Appropriate hospital referral within 24 hours of blister formation can significantly decrease infection rates and hospital stays.⁶ In addition to fluid resuscitation and nutritional support, the main goals when treating TEN are to protect the exposed areas, encourage new skin growth and prevent infections. Key components of wound care include:

• Gentle cleaning and removal of dead skin:
  • Removing damaged skin helps prevent infections, but must be done carefully. Medical professionals often use saline-soaked cloths or gentle washes to clean wounds without causing further damage⁹
  • The benefit of surgical debridement (the surgical removal of damaged tissue) in TEN care remains unclear with some surgeons arguing its benefits in lowering mortality rates and some professionals preferring “anti-shear” therapy, where, after blister fluid is removed, dead skin is left in place to act as a biological dressing⁴
• Specialised dressings:
  • Non-sticky dressings such as silicon-coated mesh or hydrocolloid sheets are used to cover wounds. These reduce pain from friction and allow the skin to heal underneath¹⁰
  • Advanced materials like Biobrane, a biosynthetic dressing, or natural coverings like amniotic membranes can promote faster healing and reduce discomfort¹⁰
  • Keeping the wounds moist with these dressings prevents them from drying out and cracking
• IV fluid to prevent dehydration:
  • Fluid replacement should be determined by urine output, aiming for 0.5–1 mL/kg/h⁴
• Nutritional support:
  • Healing large wounds requires extra energy and proper nutrition. Enteral or tube feeding should be started as early as possible if necessary⁴
• Pain Management:
  • Open wounds can be extremely painful, therefore patients may need strong painkillers or topical anaesthetics. Ensuring comfort is an important part of would-care

Infection prevention 

Infections and organ failure are the leading causes of death in TEN.⁹ Without the skin’s protective barrier, pathogens can easily invade, leading to severe infections like sepsis. Steps to minimise infection include:

• Maintaining a clean environment:
  • Medical staff follow strict hygiene practices, including the use of sterile gloves and equipment Patients are often kept in isolated, controlled environments to reduce exposure to pathogens (germs)
• Antibiotic use:
  •  Signs of infection are closely monitored. While preventive antibiotics are not always given, they may be used if there is a high risk or early signs of infection⁴
• Topical antimicrobials:
  • Applying creams or ointments with ingredients like cyclosporine and silver sulfadiazine can help control bacterial growth⁴
  • Corticosteroids, Intravenous immunoglobulin (IVIg), plasmapheresis and tumour necrosis factor (TNF)-alpha inhibitors are sometimes also used in treatment regimens^4,11
• Monitoring for infection:
  • Medical teams regularly check wounds and test blood for signs of infection. Early detection and treatment of infections are crucial to patient survival.

Multidisciplinary care

TEN patients often need care from many types of specialists, including dermatologists, burn experts and critical care doctors. Nurses, however, are the key to effective care as TEN patients present with a unique range of nursing care needs.⁷ Getting patients to specialised burn centres can make a significant difference. These facilities have the equipment and expertise needed to provide the best care.

• As multiple organ systems can be affected, anaesthesiologists, physiotherapists, nutritionists, ear, nose, and throat specialists, dermatologists, ophthalmologists, colorectal surgeons, colorectal surgeons, gynaecologists and urologists should be consulted⁵
• Support for the whole body:
  • TEN can cause fluid loss, temperature instability, and problems with electrolytes. Managing these issues is as important as wound care

Innovations in treatment

Research is continually improving how TEN is treated. Some promising advancements include:

• Stem cell therapy:
  • Early studies suggest that stem cells may help repair damaged skin and reduce inflammation¹²
• New dressings:
  • Scientists are developing dressings with built-in healing agents, like growth factors or infection-fighting peptides¹¹
  • Modern dressings such as nanocrystalline dressings can be kept in situ for longer and are thus preferred over traditional dressings⁵ 
• Better diagnostics:
  • Proteins such as granulysin may help professionals predict how severe a TEN case will become, enabling faster, more targeted treatments¹²

Challenges in TEN care

• Recognizing TEN early:
  • Fast identification and stopping the triggering medication are critical. Delays can worsen outcomes
  • Policies should be put in place for hospitals with burns units to be able to quickly identify TEN and react promptly to prevent serious complications^5,9
• Access to specialized care:
  • Not all patients have access to burn units or advanced therapies, particularly in low-resource settings
• Preventative treatments:
  • Future treatments may use genetic testing to identify who is at risk for TEN and prevent it altogether

Supportive care for complications

TEN affects more than just the skin. Patients often need care from a team of specialists to address issues such as:

• Persistent skin infections, discolouration and scarring
• Issues with organs such as the liver, lungs and kidneys
• Eye problems such as persistent inflammation, dryness and sight problems
• Issues with genitals/urinary system due to scarring

The psychological impact of TEN

Experiencing TEN can be deeply traumatic. The pain, isolation, and appearance changes can lead to anxiety, depression or post-traumatic stress. Mental health support, including therapy or counselling, is an essential part of recovery.

Individuals recovering from TEN may also need help adjusting to long-term changes, such as scarring, vision or mobility problems. Education on how to care for the skin and avoid triggering medications is vital to prevent future episodes.

Summary

Caring for someone with Toxic Epidermal Necrolysis requires a combination of advanced medical techniques, careful monitoring and compassionate support. By focusing on wound care and infection prevention, medical teams can give patients the best chance of recovery. Ongoing research offers hope that outcomes for this devastating condition will continue to improve.

Frequently asked questions

What is the cause of toxic epidermal necrolysis?

TEN is primarily caused by a reaction to certain drugs such as antibiotics, anticonvulsants, sulfa drugs and allopurinol. TEN has been linked to infections and even genetic inheritance. TEN can also be idiopathic (no known cause).

How quickly does TEN develop?

TEN typically develops 1 to 4 weeks after exposure to the trigger.

How do you treat TEN syndrome?

TEN is treated in a hospital, usually in a burns unit or intensive care unit trained to treat similar conditions. Supportive care such as pain management, wound care, infection prevention and support with nutrition and fluid all contribute to recovery, which can take anywhere from several weeks to months. 

What is the survival rate for TEN?

TEN is extremely rare but has a very high mortality rate of 14.8 to 48%.⁴

What are the symptoms of TEN?

TEN usually starts with fever and body aches resembling flu-like symptoms. Red, painful skin patches then develop and quickly worsen. The patches are dark in the middle and lighter around the outside. The rash usually starts on the upper torso and then spreads to the face, arms, legs and other areas of the body such as the mouth/throat, eyes or genitals/urethra/anus. These patches can form blisters and peel away, leaving areas that resemble severe burns. 

What dressings are used for toxic epidermal necrolysis?

Various dressings can be used for TEN including biological dressings (e.g., allograft, homograft and xenograft), biosynthetic dressings (e.g., biobrane) or silver-impregnated dressings.