What is Stevens-Johnson syndrome?

Stevens-Johnson syndrome is a rare, serious disorder of your skin and mucous membranes. It's usually a reaction to a medication or an infection. Often, Stevens-Johnson syndrome begins with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters. Then the top layer of the affected skin dies and sheds.

Stevens-Johnson syndrome is a medical emergency that usually requires hospitalization. Treatment focuses on eliminating the underlying cause, controlling symptoms and minimizing complications.

Recovery after Stevens-Johnson syndrome can take weeks to months, depending on the severity of your condition. If it was caused by a medication, you'll need to permanently avoid that drug and others closely related to it.

Stevens-Johnson syndrome symptoms include:

• Facial swelling
• Tongue swelling
• Hives
• Skin pain
• A red or purple skin rash that spreads within hours to days
• Blisters on your skin and the mucous membranes of your mouth, nose, eyes and genitals
• Shedding of your skin

If you have Stevens-Johnson syndrome, several days before the rash develops you may experience:

• Fever
• Sore mouth and throat
• Fatigue
• Cough
• Burning eyes

When to see a Doctor

Stevens-Johnson syndrome requires immediate medical attention. Seek emergency medical care if you experience any of the following signs or symptoms:

• Unexplained widespread skin pain
• Facial swelling
• Blisters on your skin and mucous membranes
• Hives
• Tongue swelling
• A red or purplish skin rash that spreads
• Shedding of your skin

Stevens-Johnson syndrome is a rare and unpredictable reaction. Your doctor may not be able to identify its exact cause, but usually the condition is triggered by a medication or an infection.

Medication and Therapy Causes

Drugs that can cause Stevens-Johnson syndrome include:

• Anti-gout medications, such as allopurinol
• Pain relievers such as acetaminophen (Tylenol, others), ibuprofen (Advil, Motrin IB, others) and naproxen sodium (Aleve)
• Medications to fight infection, such as penicillin
• Medications to treat seizures or mental illness (anticonvulsants and antipsychotics)
• Radiation therapy

Infectious causes

Infections that can cause Stevens-Johnson syndrome include:

• Herpes (herpes simplex or herpes zoster)
• Pneumonia
• HIV
• Hepatitis

Risk Factors

Factors that increase your risk of developing Stevens-Johnson syndrome include:

• Viral infections: Your risk of Stevens-Johnson syndrome may be increased if you have an infection caused by a virus, such as herpes, viral pneumonia, HIV or hepatitis.

• Weakened immune system: If you have a weakened immune system, you may have an increased risk of Stevens-Johnson syndrome. Your immune system can be affected by an organ transplant, HIV/AIDS and autoimmune diseases, such as lupus.

• A history of Stevens-Johnson syndrome: 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 Stevens-Johnson syndrome: If an immediate family member has had Stevens-Johnson syndrome or a related condition called toxic epidermal necrolysis, you may be more susceptible to developing Stevens-Johnson syndrome too.

• Genetically:  If you have a gene called HLA-B 1502, you have an increased risk of Stevens-Johnson syndrome, particularly if you take certain drugs for seizures or mental illness. Families of Chinese, Southeast Asian or Indian descent are more likely to carry this gene.

REMEMBER: 

Stevens-Johnson syndrome is an emergency medical condition. If you have signs and symptoms, call for emergency medical help, or go to an emergency room immediately.

If you have time before you go:

1. Put in a plastic bag all the medications you've taken in the last three weeks, including prescription and over-the-counter (nonprescription) drugs. Take the bag with you, as it may help the doctor figure out what triggered your condition.
2. Ask a family member or a friend to come along, if he or she is available immediately. You may want to share relevant health information about yourself with him or her, so this person can help you when you talk with your doctor.