Toxic Epidermal Necrolysis

What is toxic epidermal necrolysis?

Toxic epidermal necrolysis is a severe and potentially life-threatening skin condition that develops rapidly, often within 1 to 3 days of exposure to the triggering factor. As a serious skin reaction, it causes widespread redness, blistering, and peeling of the skin in sheets, leaving large raw areas exposed. This extensive skin loss can lead to massive fluid and electrolyte imbalances, making patients acutely ill and at high risk for infection and organ failure. The severity of this skin condition is emphasized by its high mortality rate, which can reach 25-30% in adults. Due to the rapid progression and potential for severe complications, immediate medical attention and treatment in a specialized facility, such as a burn unit or intensive care unit, is crucial for managing this devastating skin reaction.

Toxic epidermal necrolysis is a serious, potentially life-threatening skin condition characterized by redness, severe blistering, and widespread skin detachment and peeling. Toxic epidermal necrolysis can spread rapidly and affect greater than 30 percent of the body.

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See photos of toxic epidermal necrolysis here.

Beyond skin-specific symptoms, those with this condition may also experience fever, burning/stinging eyes, and discomfort with swallowing.

Treatments include discontinuing harmful drugs or medication, seeking aid from the burn unit or intensive care unit, plasma exchanges, or intravenous human immune globulin.

You should be brought to the ER as soon as possible for immediate medical treatment.

Toxic epidermal necrolysis symptoms

Toxic epidermal necrolysis presents with a few non-specific symptoms initially, followed by skin-related symptoms.

Non-specific symptoms

The serious full-body rash that occurs in toxic epidermal necrolysis is often preceded by non-specific symptoms such as:

• Fever
• Burning/stinging eyes
• Swallowing discomfort

Skin-related symptoms

The rapid skin peeling, damage and oozing happens variably from person to person and can occur in hours or days after initial onset. The main symptoms result in the following:

• Painful, red area that spreads quickly: These areas will appear as angry, red patches of skin.
• Skin peeling without blistering: The skin will begin to peel like a sunburn but in large pieces that should be concerning.
• Raw areas of skin with blisters: During the course of the disease, the skin rapidly fuses together and becomes tense, painful, large blisters.
• Mucosal involvement: The rash spreads rapidly to various linings of the body that secrete mucus/fluid such as the genital areas, eyes, mouth and throat.

Causes of toxic epidermal necrolysis

The exact mechanism behind toxic epidermal necrolysis is not known; however, it is thought to be due to an intense immune response primarily to certain drug classes. In most cases of toxic epidermal necrolysis, there is a strong, direct association between drug consumption followed by onset of symptoms and disease.

Drug types

Drug types that have been identified as likely triggers of toxic epidermal necrolysis include:

• Antibiotics:This class of medications is used to treat bacterial infections.
• Anticonvulsants: This class of medications is used to primarily treat seizures. Anticonvulsants are sometimes also used to treat general nerve pain or numbness.
• Non-steroidal anti-inflammatory drugs: More commonly referred to as NSAIDs, non-steroidal anti-inflammatory drugs relieve or reduce pain by reducing widespread bodily inflammation. The most common examples of this class are aspirin and ibuprofen.
• Xanthine oxidase inhibitors: This is a class of medications used to treat high levels of uric acid in the body. The most common and well-known drug in this class is called allopurinol which lowers the amount of uric acid in the body and prevents gout attacks.

Treatment options and prevention

The amount of skin involvement in toxic epidermal necrolysis is very important in deciding how well treatment will work. Furthermore, a skin biopsy is important in diagnosing toxic epidermal necrolysis because it is necessary to rule out a similar diagnosis.

Treatment

Treatments for toxic epidermal necrolysis include:

• Discontinue the suspected drug: The drug in suspect should be discontinued immediately as it is the trigger of the condition.
• Burn unit/intensive care unit: People need a lot of one-on-one support and observation, especially to prevent infection and to care for the skin in a similar way to individuals affected byburns.
• Plasma exchange: This is a possible therapeutic option that removes your blood and separates the plasma from the blood cells and platelets and replaces it. This procedure is thought to work because the removed plasma may contain drugs or antibodies (immune system proteins) that may be causing the disease.
• Intravenous human immune globulin (IVIG): Providing immune system proteins may prevent further damage by blocking antibodies that are causing the response.

Prognosis

In toxic epidermal necrolysis, the death rate can be as high as 25 percent in adults and can be even higher in older adults with very severe blistering.

If the person affected survives, the prognosis can be positive. The skin grows back on its own, and, unlike burns, skin grafts are not needed. Fluids and salts, which are lost through the damaged skin, are replaced intravenously.

When to seek further consultation

As soon as you notice any non-specific symptoms such as fever or burning/stinging eyes after ingestion of any of the medication classes, be on high alert. If you notice any signs of a rash, go to the emergency department immediately.