What Is Stevens-Johnson Syndrome?
Stevens-Johnson syndrome, also called SJS, is a rare but serious problem. Most often, it's a severe reaction to a medicine you've taken. It causes your skin to blister and peel off. It affects your mucous membranes, too. Blisters form inside your body, making it hard to eat, swallow, even pee.
Getting treated right away helps protect your skin and other organs from lasting damage.
SJS vs. TEN
Stevens-Johnson syndrome is similar to a condition called toxic epidermal necrolysis, or TEN. TEN also causes your skin to slough off, and it's often a reaction of your immune system to a medication. It also can be caused by an infection, tumor, or vaccine.
The main difference between SJS and TEN is scale. SJS usually affects 10% or less of your body's surface. TEN affects 30% or more.
TEN is a life-threatening illness. About 25% of cases are fatal. SJS is much less lethal. The fatality rate is between 1% and 5%.
Symptoms of Stevens-Johnson Syndrome
SJS usually starts with a fever and feeling like you have the flu. A few days later, other symptoms appear, including:
- Painful red or purple skin that looks burned and peels off
- Blisters on your skin, mouth, nose, and genitals
- Red, painful, watery eyes
The SJS rash is sometimes described as looking like a target: concentric circles that are lighter around the edges and darker in the center.
SJS is dangerous. If you have these symptoms, go to the emergency room.
Stevens-Johnson Syndrome Causes
More than 100 drugs can cause SJS. Some of the most common are:
- Medicines for gout, a painful form of arthritis – especially allopurinol (Aloprim, Zyloprim)
- Pain relievers such as acetaminophen, ibuprofen, and naproxen sodium
- Sulfa antibiotics, a kind of drug that fights infections (including Bactrim and Septra)
- Medicines that treat seizures or mental illness
The medicines most likely to cause problems in kids are sulfa antibiotics, Tylenol, and drugs that treat seizures, especially carbamazepine (Carbatrol, Tegretol).
If you're going to get SJS, it will most likely happen in the first 2 months you're taking a drug.
An infection, like pneumonia or the herpes virus that causes cold sores, can also trigger SJS. This happens more often with kids than adults.
Graft-versus-host disease, which you can get after a stem cell transplant, also can lead to SJS.
You're more likely to get SJS if you have:
- HIV or other problems with your immune system, such as lupus
- Had SJS before
- Certain genes you inherit from your parents, especially a variation of a gene called human leukocyte antigen-B
- Radiation treatments
- Had a bone marrow transplant
Sometimes, doctors can't pinpoint any specific cause for Stevens-Johnson syndrome.
Stevens-Johnson Syndrome Diagnosis
To figure out whether you have Stevens-Johnson syndrome, your health care provider will start by asking questions about your medical history, including any medications you've recently started or stopped taking. They'll also take a look at your rash. You may need more tests. They include:
- Skin biopsy, which involves removing a small sample of your skin. It will go to a lab to be tested.
- Culture, which involves removing skin, tissue, or fluid to send for testing. This can rule out infections that could be causing your condition.
- Imaging of your lungs to check for pneumonia, if your symptoms suggest it's causing your SJS
- Blood test, which involves taking a sample of your blood. It then goes to a lab to check for signs of infection.
Your health care team also will look at your pain level, how fast the rash has spread, and how much of your skin is affected.
Stevens-Johnson Syndrome Treatment
You'll be treated for SJS in the hospital by a special team of doctors and nurses. Some people are treated in a burn center or intensive care unit.
The first thing doctors will do is to stop the medication or treat the infection that made you sick. They'll also try to relieve your symptoms, prevent infections, and support your healing.
Replace fluids and nutrients
Your body needs to stay hydrated, and your skin needs protein to rebuild. You'll probably get fluids from an IV at first, then be fed through a tube that goes into your stomach through your nose.
Wound care
Hospital staff will keep your skin clean. They'll gently remove dead skin and cover bare patches with a special dressing.
Eye care
Your care team will clean your eyes and use special drops and creams to keep them from drying out.
You could be in the hospital from 2 to 4 weeks. It takes time to recover from SJS, and most people do.
Sometimes, SJS has effects that will show up years after you heal, including:
- Scars where your skin peeled
- Infections in your gums or mouth
- Lung problems such as bronchitis, which causes a bad cough and trouble breathing
Stevens-Johnson Syndrome Complications
Complications of SJS can include:
- Eye problems, including dry eyes, sensitivity to light, and long-term issues with your vision
- Pneumonia
- Shock
- Sepsis, which occurs when your entire body has an inflammatory reaction to an infection
- Multiple organ failure, in which more than one of your vital organs stop working properly. Those organs include your liver, kidneys, heart, brain, lungs, and small intestine.
In the most serious cases, Stevens-Johnson syndrome can be fatal, but that's a relatively rare outcome.
Other issues can crop up after you've recovered from SJS. Those longer-term problems might include:
- Sweating more than normal
- Dry or itchy skin
- Hair loss
- Losing your nails, or nails that are misshapen
- Changes to your sense of taste
- Dry mucous membranes that make it hard to pee
- Chronic fatigue
- Lung problems such as asthma or COPD
Stevens-Johnson Syndrome Recovery
Unless you get an infection, your skin will begin to heal in 2 to 3 weeks.
You'll probably feel tired after you leave the hospital, and it might be weeks or months before you're feeling back to normal.
If a medicine caused your SJS, you can't take it ever again.
Stevens-Johnson Syndrome Prevention
There's often no way to know how you'll react to certain medicines – even ones your doctor prescribes. If you're of Asian descent, you may have a gene that raises your risk of SJS. Talk to your doctor about getting tested for this gene before you take carbamazepine.
If you've already had SJS, you can take steps to avoid getting it again:
- Tell your doctors you've had SJS.
- Wear a medical alert bracelet.
- Know the name of the medicine that caused your SJS. Avoid taking it or any drugs like it.
Takeaways
Stevens-Johnson syndrome starts out feeling like flu symptoms. Then you get a rash that blisters, turns into sores, and causes your skin to peel. It can affect your mucous membranes, too. It's usually a severe reaction to a medication. SJS is a dangerous disease that can have serious complications; it can be fatal. If you have it, you'll spend time in the hospital. Most people recover, although some have long-term complications.
Stevens-Johnson Syndrome FAQs
How serious is Stevens-Johnson syndrome?
Stevens-Johnson syndrome is rarely fatal. But it's a serious disease that has to be treated in a hospital. You may need to be in intensive care or a burn unit. Almost everyone recovers from SJS, but some people have long-term complications that affect their skin, eyes, and lungs.
How fast does Stevens-Johnson syndrome progress?
SJS starts with a flu-like illness. Your eyes may burn, too. Then, in 1 to 3 days, a rash will appear on your skin and on your mucous membranes. With treatment, your skin will heal in 2 to 3 weeks. SJS can start up to 2 weeks after you've stopped taking the medication that triggered it.
What can be mistaken for Stevens-Johnson syndrome?
Some autoimmune diseases can cause your skin to blister, and that condition can look like SJS. It might also be confused with something called erythema multiforme major, another type of allergic reaction to medicine or infection.
