What is Stevens Johnson Syndrome

Stevens Johnson syndrome is a disorder of the mucous membranes and skin. This disorder causes these things to happen:

• The mucous membranes become inflamed.
• The top layer of skin dies and starts to shed. The more skin that dies, the more serious the disorder becomes.
• The body loses fluids quickly.
• The body loses its ability to keep germs out.

This condition requires immediate treatment to prevent complications such as:

• Too much fluid loss.
• Blood infection.
• Eye damage.
• Skin damage and infection.
• Vision loss, if the eyes are affected.
• Damage to the lungs, heart, kidneys, or liver.

What are the causes?

The most common cause of this condition is an allergic reaction to a medicine. Medicines that are known to cause this condition include:

• Antibiotic medicines.
• Antiseizure medicines.
• Medicine that is used to treat gout.
• Cocaine.
• NSAIDs.

This condition can also be caused by an infection. In some cases, the cause may not be known.

What increases the risk?

This condition is more likely to develop in:

• People who have a variation in the HLA gene. This variation may be passed down through families (inherited).
• People who have a family history of Stevens-Johnson syndrome.
• People who have cancer or are having cancer treatment.
• People who have a weak body defense system (immune system).
• People who have systemic lupus erythematosus.
• People of Asian, Chinese, or Indian descent.

What are the signs or symptoms?

This condition often begins with several days of flu-like symptoms, such as:

• Fever.
• Sore throat.
• Fatigue.
• Headache.
• Muscle aches.
• Dry cough.
• Burning feeling in the eyes.

Then, a painful red or purple rash may develop on the face, trunk, palms, or soles, and spread to other parts of the body. The rash creates blisters and open sores on the skin. If the mucous membranes are affected, the rash may be in:

• The mouth.
• The nose.
• The eyes.
• The genitals.
• The digestive tract.
• The urinary tract.

Other signs and symptoms include:

• Shedding of the skin or mucous membrane.
• Swelling of the tongue and face.
• Swelling and itching of the skin (hives).
• Redness, sensitivity to light, and dryness in the eyes.
• Pain in the mouth and throat.
• Pain when passing urine.
• Pain when swallowing.

How is this diagnosed?

This condition is diagnosed with a physical exam. Your health care provider may also do tests, such as:

• A biopsy. This involves removing a sample of skin or eye tissue to be looked at under a microscope.
• Blood tests
• Imaging tests.

If you are having any eye symptoms, you may need to be seen by an eye specialist (ophthalmologist).

How is this treated?

This condition may be treated by:

• Stopping medicines that you are currently taking.
• Getting fluids and nourishment through an IV tube or through a tube that is passed through your nose and into your stomach (nasogastric tube).
• Gently removing dead skin and putting a moist bandage (dressing) on those areas.
• Applying eye drops or having eye surgery.
• Using a mouthwash that numbs the mouth and throat to help with swallowing.
• Medicines:
  • To help you relax (sedatives).
  • To control your pain.
  • To fight infection (antibiotics).
  • To stop skin swelling and itching.

Follow these instructions at home:

Medicines

• Take over-the-counter and prescription medicines only as told by your health care provider.
• If you were prescribed an antibiotic medicine, take it as told by your health care provider. Do not stop taking the antibiotic even if you start to feel better.
• If a medicine triggered your condition, talk with your health care provider before you take the medicine again. Do not take it if your health care provider tells you not to.
• Do not start taking any new medicines before you ask your health care provider if they are safe for you.

Other Instructions

• Tell all of your health care providers that you have had Stevens-Johnson syndrome. If the condition was caused by a medicine, always tell your health care providers which medicine caused it.
• Wear a medical bracelet or necklace that says that you had this condition and tells the cause of it.
• Ask your health care provider if you should be tested for the HLA gene.
• Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if:

• You have trouble managing complications of the condition.

Get help right away if:

• You have flu-like symptoms after you have an infection or after you start a new medicine.
• You develop symptoms on your skin or mucous membranes again.