What Is Toxic Epidermal Necrolysis (TEN)?

Toxic epidermal necrolysis (TEN) is a rare but potentially life-threatening condition that causes a severe reaction in the skin and mucous membranes. It’s usually triggered by taking certain medications, but infection can occasionally cause this reaction. TEN is a severe form of Stevens-Johnson syndrome (SJS).

This article will review toxic epidermal necrolysis, its causes, symptoms, and how it’s treated. 

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What Causes Toxic Epidermal Necrolysis?

TEN is a condition that occurs when the immune system overreacts when exposed to a certain trigger, usually medications or infection.

Although not fully understood, it is thought that immune system cells, specifically T cells, which fight off invaders such as viruses and bacteria, attack the cells of the skin and mucous membranes. This causes severe inflammation, blistering, and tissue death. 

Drug-Induced

The most common triggers of TEN are medications. They are estimated to be the cause in 50% to 95% of TEN cases. Certain classes of medications are more likely to cause TEN than others, and include over 100 potential medications.

Medications include:

• Anti-seizure drugs: Tegretol (carbamazepine), Lamictal (lamotrigine), Dilantin (phenytoin)
• Antibiotics: Sulfonamides, penicillin, cephalosporins, vancomycin
• Nonsteroidal anti-inflammatory drugs (NSAIDs): Advil or Motrin (ibuprofen), Aleve (naproxen)
• Zyloprim (allopurinol)
• Viramune (nevirapine)

Although many of these medications are commonly used, TEN is rare. It’s estimated that TEN occurs in 1 to 1.5 out of every 1 million people in a year.

Infection

Although not as common a cause as medications, infections can trigger the development of TEN as well. Mycoplasma pneumoniae, a type of bacteria that causes walking pneumonia (a mild case of pneumonia), is the most common infectious trigger of TEN.

Other infections include:

• Tuberculosis
• Syphilis
• Chlamydia
• Salmonella
• Enterobacter
• Streptococci

How Does TEN Impact Your Health?

TEN is a serious condition and is potentially life-threatening. Symptoms can begin four to 28 days after the triggering event, either a medication or an infection. Before skin reactions develop, flu-like symptoms appear. These symptoms include:

• Fever
• Sore throat
• Headaches
• Joint pain
• Fatigue

When skin changes develop, they can be widespread across the body and progress rapidly. In addition to blistering and skin peeling, the skin can become red and feel painful and raw. It may start to slough off in large amounts, leaving open wounds similar to burns.

Mucous membranes of the eyes, mouth, throat, and genitals are also frequently affected. The eyes can become red and irritated, and the cornea of the eye can become damaged as that tissue is affected. Light sensitivity is common.

The mouth can develop painful ulcers and sore throat, which make eating and drinking difficult. The airway membranes can also be affected, leading to shortness of breath and cough.

The skin protects against infection and plays an important role in fluid and electrolyte balance in the body. With TEN, over 30% of the skin on the body's surface is affected. This can lead to severe complications, which can include:

• Severe infection (sepsis)
• Dehydration
• Pneumonia (lung infection and inflammation)
• Organ failure
• Death

TEN vs. Stevens-Johnson Syndrome

TEN and SJS are both serious conditions that are similar and involve skin and mucous membrane detachment. However, TEN is more severe, with over 30% of the body surface area affected. A diagnosis of SJS is made when less than 10% of the body surface area is affected.

How Toxic Epidermal Necrolysis Is Diagnosed

The first step in diagnosing TEN is a physical examination by a healthcare provider. If someone presents with widespread skin peeling, blistering, and redness, which also involves the mucous membranes, they may suspect TEN. However, a skin biopsy (taking a sample to analyze in the lab) may be needed to rule out another cause.

A detailed medical history is necessary. This can help determine any recent medications used, when the symptoms started, and if anything like this has happened previously. Determining the cause, if possible, is crucial so the medication can be stopped immediately or the infection can be treated. 

Risk Factors for Developing Toxic Epidermal Necrolysis

The most significant risk factor for developing TEN is being exposed to a medication that causes it. Over 100 medications can be the culprit. However, some people may have genetic factors that put them at a higher risk of developing TEN.

Some people may be born with an alteration in the HLA-B gene. This gene helps the immune system identify an invading organism vs. the body’s own tissue. Some mutations of this gene can cause the immune system to overreact and attack the skin and mucous membranes if exposed to certain medications or infections.

Although TEN can occur at any age, it is most likely seen in people over 40.

Can You Recover From TEN?

TEN is a medical emergency, and has a mortality rate of 30% to 40%.

Recovery from TEN is possible. It can take a long time, it requires intense medical care, and you will likely need a prolonged hospitalization. A person’s ultimate outlook on recovery can vary based on many factors, including their overall health, the severity of TEN, and any complications that may have occurred.

Long-term effects from TEN can include:

• Permanent skin discoloration
• Dry mouth
• Dry skin
• Hair loss
• Changes to the nails, including loss of the nails
• Excessive sweating

Toxic Epidermal Necrolysis Treatments

Treating TEN requires hospitalization and treatment from multiple healthcare teams. Care is often given in a hospital’s intensive care or burn unit.  

The initial treatment is to stop the medication or treat the infection causing TEN, if known. Supportive care is the mainstay of treatment and includes:

• Fluid and electrolyte management
• Temperature management
• Pain management
• Nutritional support
• Wound care
• Prevention of and monitoring for infection
• Frequent monitoring of fluid status, vital signs

Medications such as immunosuppressants and steroids might be used. However, there is no current standard of care for medications to treat TEN.

When to Contact a Healthcare Provider

TEN is a medical emergency. If you suspect you have TEN, call 911. The sooner TEN can be treated, the better the outcome. 

Summary 

Toxic epidermal necrolysis is a condition in which the immune system overreacts to a trigger, usually a medication or infection, and causes damage to the tissues of the skin and mucous membranes. This causes the skin to blister and peel. It can lead to severe complications such as infection, organ failure, and death.

TEN is treated with supportive care, which includes hospitalization and monitoring for complications. If someone suspects they may have TEN, they should call 911 for emergency treatment.