Toxic Epidermal Necrolysis (TEN)

What is toxic epidermal necrolysis?

Toxic epidermal necrolysis (TEN) is a painful, life-threatening skin condition. It causes large areas of blistering and peeling skin on at least 30% of your body, including mucous membranes like the mouth, eyes and genitals. It's the result of a reaction to certain medications. Widespread damage to the skin can lead to dangerous fluid loss and infections.

TEN, or TEN syndrome, is a rare, severe form of Stevens-Johnson syndrome (SJS). SJS causes blistering and peeling skin on less than 10% of the body. The two conditions overlap when there is 10% to 30% body coverage.

Who is at risk of getting toxic epidermal necrolysis?

People with weakened immune systems are more likely to develop SJS or TEN. Immune system problems can be the result of:

• Human immunodeficiency virus (HIV).
• Lymphoma.

Genetics may also play a role. Some people cannot normally break down certain medications, which can cause their body to develop Stevens-Johnson syndrome or toxic epidermal necrolysis. Mutations (changes) to certain genes may make individuals (or ethnic groups) more likely to react to particular medications.

People of any age can get TEN, though it’s more common in older populations.

How common is toxic epidermal necrolysis?

TEN is a rare disease. Together, SJS and TEN affect about 1 million to 2 million people each year.

What causes toxic epidermal necrolysis?

In most cases, experts think that certain medications trigger TEN syndrome. These medications may include:

• Allopurinol (drug used to treat kidney stones and gout).
• Anticonvulsants (group of drugs used to treat seizures).
• Anti-retroviral drugs used to treat HIV (especially NNRTIs like nevirapine, efavirenz, or etravirine).
• Oxicams (a group of non-steroidal anti-inflammatory drugs or NSAIDs).
• Sulfonamides (a type of antibiotic).

Other rare triggers may include:

• Infections
• Vaccinations.

There are some cases where the cause of TEN is not known.

What are the symptoms of toxic epidermal necrolysis?

TEN symptoms may develop a few weeks after you start taking a medication. It usually begins with flu-like symptoms, including:

• Body aches.
• Cough.
• Fever and chills.
• Headache.
• Redness and inflammation in the eyes.

TEN then causes a painful skin rash before progressing to large areas of blistering and peeling skin. Erosions, or painful open wounds that look like burns, develop as the skin peels away.

Erosions tend to start on the face and chest. They often spread to the following areas of the body:

• Eyes, including the conjunctiva (inside of the eyelid) and cornea (outer lens of the eye).
• Mucous membranes, including inside the mouth, nose, throat and airways.
• Urinary tract, anus and genitals.

Erosions can make it difficult and painful to eat, swallow, breathe, see, urinate and perform other functions.

What are the complications of toxic epidermal necrolysis?

Life-threatening complications of TEN syndrome can include:

• Infections.
• Multiple organ failure.
• Pneumonia.
• Sepsis.

How is toxic epidermal necrolysis diagnosed?

Your healthcare provider can usually diagnose TEN after evaluating your skin and symptoms. In some cases, you may need a skin biopsy to confirm the diagnosis. A pathologist (healthcare provider who examines body tissues) studies a sample of your skin under a microscope. The biopsy shows:

• Necrotic (dead) skin cells.
• Detachment of the first layer of skin (epidermis) from the second layer.

How is toxic epidermal necrolysis treated?

TEN requires emergency medical treatment at a hospital. You’ll receive care in an intensive care unit (ICU) or burn center. Immediate treatment includes:

• Stopping all medications that could be triggering TEN.
• Replacing lost body fluids through an IV.
• Preventing skin infections with antibacterial creams or ointments.
• Giving antibiotics at the first sign of infection.

Other treatments for TEN may include:

• Immune globulin: You receive immune globulin through an IV. It’s a sterile liquid made from donor plasma (the liquid part of the blood). It contains antibodies or substances that help your body fight infections.
• Immunosuppressants: These drugs weaken the immune system when it isn’t working right. They keep your body from attacking its own tissues.
• Plasmapheresis: This procedure removes blood from your body and separates the plasma from blood cells. A machine removes substances causing a harmful immune response from the plasma. Then, the machine returns the “clean” blood or a plasma substitute to your body.

How can I prevent toxic epidermal necrolysis?

You may be able to prevent TEN by seeking immediate medical care at the first signs of Stevens-Johnson syndrome.

What is the outlook for people with toxic epidermal necrolysis?

About 25% of people who develop TEN don’t survive. For those who do survive, the erosions usually heal and skin grows back on its own. Most people don’t need skin grafts.

Does toxic epidermal necrolysis cause long-term effects?

People who survive TEN may experience a wide range of long-term effects. Talk to your doctor about your specific symptoms, which may show up in your:

Skin, hair and nails:

• Abnormal nail growth or nail loss.
• Abnormal pigmentation (patches of skin that are lighter or darker than the surrounding skin).
• Alopecia areata (hair loss).
• Dry or itchy skin.
• Hyperhidrosis (excessive sweating).

Eyes:

• Chronic (ongoing) eye inflammation or red eye.
• Dry eyes.
• Eyelid abnormalities.
• Sensitivity to light.
• Vision loss or, rarely, blindness.

Mucous membranes of the mouth, nose, throat and airways:

• Bronchitis.
• Chronic obstructive pulmonary disease (COPD).
• Dry mouth.
• Gum disease.
• Loss of taste or smell.
• Lung damage.

Urinary tract, anus and genitals:

• Chronic erosions or inflammation in the urethra, vagina or penis.
• Difficulty urinating.
• Vaginal dryness.

A note from Cleveland Clinic

Toxic epidermal necrolysis (TEN) is a rare, life-threatening skin condition. It’s the most severe form of Stevens-Johnson syndrome. TEN is usually the result of your body’s immune system reacting to a medication. It causes painful, blistering, peeling skin on at least 30% of the body, leading to open erosions. Open erosions may lead to dangerous complications such as sepsis, organ failure, infections or death. TEN requires immediate medical attention.