What Is Toxic Epidermal Necrolysis (TEN)?

Causes

Because TEN is so rare, it isn’t fully understood. It’s typically caused by an abnormal reaction to medication. Sometimes, it’s difficult to identify the underlying cause of TEN.

Medication

The most common cause of TEN is an abnormal reaction to medication. It’s also known as a dangerous type of drug rash, and is responsible for up to 95 percent of TEN cases.

Often, the condition forms within the first 8 weeks of taking the drug.

The following medications are most commonly associated with TEN:

• anticonvulsants
• oxicams (nonsteroidal anti-inflammatory drug)
• sulfonamide antibiotics
• allopurinol (for gout and prevention of kidney stones)
• nevirapine (anti-HIV drug)

Infections

In very rare instances, a TEN-like illness is linked to an infection by a bacteria known as Mycoplasma pneumoniae, which causes a respiratory infection.

Symptoms

The symptoms of TEN are different for each person. In the early stages, it usually causes flu-like symptoms. This may include:

• fever
• body aches
• red, stinging eyes
• difficulty swallowing
• runny nose
• coughing
• sore throat

After 1 to 3 days, the skin peels with or without blistering. These symptoms can progress within several hours or days.

Other symptoms include:

• red, pink, or purple patches
• painful skin
• large, raw areas of skin (erosions)
• symptoms spreading to the eyes, mouth, and genitals

Visual examples

The primary symptom of TEN is painful peeling of the skin. As the condition progresses, the peeling rapidly spreads throughout the body.

Below are visual examples of TEN.

Connection with Stevens-Johnson syndrome

Stevens-Johnson syndrome (SJS), like TEN, is a severe skin condition caused by a drug or, rarely, associated with an infection. The two conditions are on the same spectrum of disease and differ based the amount of skin involved.

SJS is less severe. For example, in SJS, less than 10 percent of the body is affected by skin peeling. In TEN, more than 30 percent is affected.

However, SJS is still a serious condition. It also requires immediate emergency medical attention.

SJS and TEN often overlap, so the conditions are sometimes referred to as Stevens-Johnson syndrome/toxic epidermal necrolysis, or SJS/TEN.

Risk factors

Though anyone taking medication can develop TEN, some people have a higher risk.

Possible risk factors include:

• Older age. TEN can affect people of all ages, but it’s more likely to affect older adults.
• Gender. Females may have a higher risk of TEN.
• Weakened immune system. People with a weakened immune system are more likely to develop TEN. This may occur due to conditions like cancer or HIV.
• AIDS. SJS and TEN are 1,000 times more common in people with AIDS.
• Genetics. The risk is higher if you have the HLA-B*1502 allele, which is most common in people of Southeast Asian, Chinese, and Indian descent. The gene can increase your risk of TEN when you take a certain drug.
• Family history. You may be more likely to develop TEN if an immediate relative has had the condition.
• Past drug reactions. If you’ve developed TEN after taking a certain drug, you have an increased risk if you take the same medication.

Diagnosis

A doctor will use a variety of tests to diagnose your symptoms. This may include:

• Physical exam. During a physical exam, a doctor will inspect your skin for peeling, tenderness, mucosal involvement, and infection.
• Medical history. To understand your overall health, a doctor will ask about your medical history. They’ll also want to know what drugs you take, including any new medications taken in the past two months, as well as any allergies you have.
• Skin biopsy. During a skin biopsy, a sample piece of affected skin tissue is removed from your body and sent to a lab. A specialist will use a microscope to examine the tissue and look for signs of TEN.
• Blood test. A blood test can help identify signs of infection or other problems with internal organs.
• Cultures. A doctor can also look for an infection by ordering a blood or skin culture.

While the doctor is usually able to diagnose TEN with a physical exam alone, a skin biopsy is often performed to confirm the diagnosis.

Treatment

In all cases, treatment includes discontinuing the drug that caused your reaction.

Other forms of treatment depend on several factors, such as:

• your age
• your overall health and medical history
• the severity of your condition
• the affected body areas
• your tolerance of certain procedures

Treatment will involve:

• Hospitalization. Everyone with TEN needs to be cared for in a burn unit.
• Ointments and bandages. Proper wound care will prevent further skin damage and protect the raw skin from fluid loss and infection. To protect your skin, your hospital team will use topical ointments and wound dressings.
• Intravenous (IV) fluid and electrolytes. Extensive burn-like skin loss, especially in TEN, leads to fluid loss and electrolyte imbalance. You’ll be given IV fluid and electrolytes to minimize the risk. Your hospital team will closely monitor your electrolytes, the status of your internal organs, and your overall fluid status.
• Isolation. Since the skin damage of TEN increases the risk of infection, you will be isolated from others and potential sources of infection.

Medications used to treat TEN include:

• Antibiotics. Almost everyone with TEN is given antibiotics to prevent or treat any infections.
• Intravenous immunoglobulin G (IVIG). Immunoglobulins are antibodies that help your immune system. IVIG is sometimes used to control the reaction. This is an off-label use of IVIG.
• TNF alpha inhibitor etanercept and immunosuppressant cyclosporine. These are promising treatments that are often recommended by experts in the treatment of TEN. This is an off-label use of both medications.

Specific body parts may need different treatments. For example, if your mouth is affected, a specific prescription mouthwash may be used in addition to other treatments.

Your hospital team will also closely monitor your eyes and genitals for signs. If they detect any signs, they’ll use specific topical treatments to prevent complications, such as vision loss and scarring.

Currently, there is no standard treatment regimen for TEN. Treatment may vary depending on the hospital. For example, some hospitals may use IVIG, while others may use a combination of etanercept and cyclosporine.

Etanercept and cyclosporine aren’t currently approved by the Food and Drug Administration (FDA) to treat TEN. However, they can be used off-label for this purpose. Off-label use means that your doctor can prescribe a drug for a condition that it isn’t approved for if they think that you may benefit from the drug. Learn more about off-label prescription drug use.

Outlook

The mortality rate of TEN is approximately 30 percent, but can be even higher. However, many factors affect your individual outlook, including your:

• age
• overall health
• severity of your condition, including body surface area involved
• course of treatment

In general, recovery can take 3 to 6 weeks. Possible long-term effects include:

• skin discoloration
• scarring
• dry skin and mucous membranes
• hair loss
• trouble urinating
• impaired taste
• genital abnormalities
• vision changes, including loss

Takeaway

Toxic epidermal necrolysis (TEN) is a serious emergency. As a life threatening skin condition, it can quickly lead to dehydration and infection. Get medical attention immediately if you or someone you know has symptoms of TEN.

Treatment includes hospitalization and admission to a burn unit. Your hospital team will prioritize wound care, fluid therapy, and pain management. It can take up to 6 weeks to get better, but early treatment will improve your recovery and outlook.