About toxic epidermal necrolysis

What is toxic epidermal necrolysis?

Toxic epidermal necrolysis (TEN) is a rare, sometimes life-threatening unless properly treated, immunological disorder of the skin. It is characterized by blisters that meld into one another to cover a substantial portion of the body (30% and more), and extensive peeling or sloughing off of skin (exfoliation and denudation). The exposed under layer of skin (dermis) is red and suggests severe scalding. Often, the mucous membranes become involved, especially around the eyes (conjunctivitis), but also the mouth, throat, and bronchial tree.

Onset can occur at any age. The infantile form frequently follows an infection. In adults the disorder is usually caused by a reaction to taking a pharmaceutical drug, especially anticonvulsants, non-steroid anti-inflammatories, and/or some antibiotics.

TEN is thought to be an immunological disorder and to be one of a family of three skin disorders. TEN is considered to be the more serious, followed by Stevens-Johnson syndrome and erythema multiforme, in order of severity of disease.

What are the symptoms for toxic epidermal necrolysis?

Early treatment is key for people with Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). If you have symptoms, seek immediate medical attention. You'll likely need care from a skin specialist (dermatologist) and other experts in a hospital.

What are the causes for toxic epidermal necrolysis?

SJS/TEN is usually caused by a skin reaction to medicine. The symptoms are likely to start showing up one to four weeks after you start taking a new drug.

The most common drug triggers of SJS/TEN include antibiotics, epilepsy drugs, sulfa drugs and allopurinol (Aloprim, Zyloprim).

What are the treatments for toxic epidermal necrolysis?

If your doctor suspects that your TEN was caused medicine you took, you'll need to stop taking that drug. Then you'll likely be moved to a hospital for treatment, possibly in its burn center or intensive care unit. Full recovery can take several months.

Supportive care

The main treatment for TEN is trying to make you as comfortable as possible while your skin heals. You'll receive this supportive care while in the hospital. It might include:

  • Fluid replacement and nutrition. Because skin loss can result in loss of fluid from the body, it's vital to replace fluids and electrolytes. You might receive fluids and nutrients through a tube inserted in the nose and guided to the stomach (nasogastric tube).
  • Wound care. Your health care team might gently cleanse the affected skin and apply special dressings infused with petroleum jelly (Vaseline) or medication. Your care team also monitors you for infection and gives you antibiotics if needed.
  • Breathing help. You might need tests and procedures to evaluate your airway and help keep it clear. With advanced disease, you might need intubation or mechanical breathing assistance (ventilation).
  • Pain control. You'll receive pain medicine to reduce your discomfort. For pain in your mouth, you might be given a mouthwash containing a numbing agent, such as lidocaine.
  • Eye care. For mild eye symptoms, you might benefit from applying preservative-free artificial tears at least four times a day. Eye drops with corticosteroids might be used to control eye inflammation. Your care team might include an eye specialist (ophthalmologist).

Medications

Treatment of TEN also might include one or a combination of medications that affect the whole body (systemic drugs), such as cyclosporine (Neoral, Sandimmune), etanercept (Enbrel) and intravenous immunoglobulin (IVIG). Further study is needed to determine their benefit, if any.

What are the risk factors for toxic epidermal necrolysis?

Factors that increase your risk of SJS/TEN include:

  • HIV infection. Among people with HIV, the incidence of SJS/TEN is about 100 times greater than that among the general population.
  • A weakened immune system. The immune system can be affected by an organ transplant, HIV/AIDS and autoimmune diseases.
  • Cancer. People with cancer, especially blood cancers (hematologic malignancies), are at increased risk of SJS/TEN.
  • A history of SJS/TEN. If you've had a medication-related form of this condition, you are at risk of a recurrence if you use that drug again.
  • A family history of SJS/TEN. If a first-degree relative, such as a parent or sibling, has had SJS/TEN, you may be more susceptible to developing it too.
  • Genetic factors. Having certain genetic variations puts you at increased risk of SJS/TEN, especially if you're also taking drugs for seizures, gout or mental illness.

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