Toxic Epidermal Necrolysis

What is toxic epidermal necrolysis?

• Toxic epidermal necrolysis (TEN) is a rare and serious condition of your skin and mucous membranes. Mucous membranes include the lining of your mouth, bowels, eyes, and nose. TEN is also called Lyell's syndrome. With TEN, you will lose your epidermis (outer layer of skin). Your skin is lost on thirty percent or more of your body. TEN can overlap with another disease, Stevens-Johnson syndrome (SJS). In SJS/TEN, you will lose between 10 to 30% of your outer layer of skin.
  
  
• With TEN, your skin may heal with or without scars. Healed skin may have pigment (color) changes. You may lose your nails and hair for your lifetime. You may have lifelong problems swallowing, and difficulty urinating. TEN may also cause you to have lifelong skin, vision, and medical problems. With TEN, you may die. Death is usually caused by infection, lung problems, or other serious medical problems.

What causes toxic epidermal necrolysis?

TEN is usually caused by a response to a medicine you have been taking. The response may happen within one week to two months of taking the medicine. TEN may also be caused by infection, vaccinations, chemicals, or diseases of your organs or whole body. The cause of TEN may be unknown, and TEN risk may be genetic (passed on by a parent). The most common medicines that may cause TEN are:

• Antibiotics (used to treat infection).
  
  
• Anti-seizure medicines (used to treat convulsions).
  
  
• Non-steroidal anti-inflammatory (NSAIDs) medicine (used to treat swelling or fever).

What are the signs and symptoms of toxic epidermal necrolysis?

For two weeks before TEN appears on your skin and mucous membranes, you may have the following symptoms:

• Anxiety.
  
  
• Cough.
  
  
• Fatigue (sleepiness).
  
  
• Fever and chills.
  
  
• Headache
  
  
• Muscle and joint pain.
  
  
• Sore throat.

TEN makes the outer layer of your skin die and fall off. You will usually see redness and blisters on your face first, then your chest. The redness and blisters will then spread to the rest of your body. You may have painful blisters and sores in your mouth, eyes, and genitals (area between your legs). It is also possible to have sores in your stomach, lungs, and colon (bowel). The blisters and sores may be very painful and feel like they are burning. When your skin dies, it may fall off in large amounts. Your skin will be dark red and look badly burned.

How is toxic epidermal necrolysis diagnosed?

Caregivers will usually know you have TEN by looking at you. Caregivers will want to know if you have felt sick, and if you have been using any medicines. By asking this, they may be able to figure out what is causing your skin problem. Your caregiver may biopsy an area of your skin to be sure it is TEN. For a biopsy, your caregiver will take a sample of your skin for testing. Your caregiver may also do other testing, such as blood tests and x-rays.

How is toxic epidermal necrolysis treated?

The goal of treating TEN is to stop symptoms from getting worse, and protect you from getting sicker. You are put in the hospital to treat TEN. Your caregiver will stop the medicine you were taking that caused the response. If your caregiver cannot learn what made you sick, he may stop all your medicines. You will need an IV put in your vein, that connects to tubing, for getting medicine and liquids. You may also be given liquid nutrition (TPN) through your IV because of protein loss. Wound (TEN sores) care will be done by your caregivers to help stop any infection from starting.

What medicines may be used to treat TEN?

• Antacids: With TEN, sores may grow in your stomach and intestinal tract and bleed. This medicine may help lower your chance of getting this kind of bleed.
  
  
• Antibiotics: Antibiotics may be given to help treat an infection (caused by germs called bacteria) in your blood. Your caregiver may also put medicine on your skin to lower your chance of infection. This medicine is a topical antibiotic or antiseptic.
  
  
• Blood thinners: This medicine helps stop clots from forming in your blood. This may be needed if you are not able to get out of bed for a long time.
  
  
• Eye drops: Eye drops may be used if you have sores in your eyes. This may help your eyes to heal, and stop infection from starting.
  
  
• Immune globulins: This medicine is given as a shot or an IV infusion to make your immune system stronger. You may need immune globulins to treat or prevent an infection. It is also used when you have a chronic condition, such as lupus or arthritis. You may need many weeks of treatment. Each infusion can take from 2 to 5 hours.
  
  
• Pain medicines: Caregivers may give you medicine to take away or decrease your pain.
  
  
• Pressors: This kind of medicine may be given to increase your blood pressure. Keeping your blood pressure in normal range protects your heart, lungs, brain, kidneys, and other organs.

What therapies and treatments are used to treat TEN?

• Hydrotherapy: This therapy is done in a whirlpool. Hydrotherapy may help to clean your wounds, and remove dead tissue from your skin.
  
  
• Hyperbaric oxygen therapy: This is also called HBO. HBO is used to get more oxygen into your body. The oxygen is given under pressure to help it get into your tissues and blood. You may be put into a tube-like chamber called a hyperbaric or pressure chamber. You will be able to see your caregivers and talk with them through a speaker. You may need to have this therapy more than once.
  
  
• Physical therapy: A physical therapist may do exercises with you. This will help your arm and leg movement if you have to stay in bed.
  
  
• Plasmapheresis: Plasma (the fluid part of blood) is taken from your blood cells and replaced with clean plasma. This is done to clean toxins out of your blood that may have caused you to have TEN.
  
  
• Surgery: Surgery may be needed if your skin does not heal right. You may need any or all of the following:
  
  
  • Debridement: This is done to clean and remove dirt, or dead tissues from the injured area.
    
    
  • Skin graft: A piece of skin is taken from one area of the body and attached to the injured area. A skin graft may help to heal your injury. Donor skin (from another person) may also be used.
  
  
• TPN: TPN stands for total parenteral nutrition. It is also called hyperalimentation. It provides your body with nutrition such as protein, sugar, vitamins, minerals, and sometimes fat (lipids). TPN is used when you have problems with eating or digesting food. TPN is usually put into your body through a large IV. You may need TPN for several days or longer.
  
  
• Wound care: Your caregivers may put special bandages on your sores to protect them, and help them heal.

What can I do to feel better when I have toxic epidermal necrolysis?

• Carefully follow your caregiver's instructions about any special diet.
  
  
• Clean your mouth as shown by your caregiver. If you have mouth sores, your caregiver may give you a special toothbrush or sponge to use. Your caregiver may also order you a special medicated mouthwash for rinsing your mouth.
  
  
• Work with your therapist to keep your arms and legs moving. This will keep you from getting too stiff from being in bed. Rest when you feel it is needed.
  
  
• Use baby shampoo to clean skin areas with hair on them.

What puts me at risk of getting toxic epidermal necrolysis?

Taking medications such as antibiotics, anti-seizure, and NSAIDs, increases your risk of getting TEN. Women are at higher risk of getting TEN than men. TEN usually happens in people over forty, but can happen in all ages. TEN also happens more in people with a poor immune system. Your body's immune system works to fight infection. If you have HIV or AIDS, your risk of getting TEN is much higher.

When should I call my caregiver?

Call your caregiver if:

• You have a fever.
  
  
• You get a rash, or sores on your mucous membranes after starting a new medicine.
  
  
• Your skin is red and hurts.

When should I seek immediate help?

Call 911 or seek immediate help if:

• You have a blistering rash on your skin, in your mouth, eyes, or genitals.
  
  
• You suddenly have trouble breathing.
  
  
• Your skin hurts and begins peeling off.

Where can I find support and more information?

Having TEN can be life changing for you and your family. Talk to your caregiver, family, and friends about your feelings. Contact the following for more information:

• Stevens Johnson Syndrome Foundation and Support Group
  P.O. Box 350333
  Westminster , CO 80035-0333
  Web Address: http://www.sjsupport.org
  

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.