Patient education: Stevens-Johnson syndrome and toxic epidermal necrolysis (The Basics)

What is Stevens-Johnson syndrome? — 

Stevens-Johnson syndrome ("SJS") is a rare but very serious problem that affects the skin. It causes the skin to turn red or purple and peel away from the body. It can affect the skin on the outside of the body, as well as the pink, moist skin that lines the mouth, nose, eyes, genitals, and other body parts.

A more severe form of SJS is called "toxic epidermal necrolysis" ("TEN"). The treatment for both conditions is very similar. Doctors often use the term "SJS/TEN" to describe both forms.

SJS/TEN is an emergency and needs to be treated in a hospital. This is explained more below.

What are the symptoms of SJS/TEN? — 

The symptoms include:

●Patches of painful, red or purple skin (picture 1) – The patches might look darker in the middle and lighter around the outside. They can also have a blister on top.

●Blisters, followed by peeling of dead skin on the face, chest, back, legs, and arms (picture 2)

●Flu-like symptoms, such as fever (often higher than 102°F, or 39°C), sore throat, body aches, and feeling very tired – These sometimes start a few days before the skin symptoms.

●Sores, swelling, and crusting on the pink, moist skin lining the mouth, eyes, vagina, penis, lungs, and other areas (picture 3 and picture 4) – This can lead to:

•The eyes getting red and watering

•Parts of the eye sticking to each other

•Pain with urination, or trouble urinating

•Trouble breathing, fluid buildup in the lungs, or lung infections

What causes SJS/TEN? — 

SJS/TEN is a rare side effect of certain medicines. It can also be caused by an infection, especially in children. Doctors cannot predict who will get SJS/TEN.

There are many medicines that can cause SJS/TEN. People are most likely to have this problem during the first 8 weeks of taking one of these medicines.

Some of the most common examples of medicines that can cause SJS/TEN include (table 1):

●A medicine used to treat gout called allopurinol (sample brand name: Aloprim)

●Medicines used to prevent seizures, pain, or other conditions, called "anti-seizure medicines." These include:

•Carbamazepine (sample brand names: Tegretol, Carbatrol)

•Phenytoin and fosphenytoin (sample brand names: Dilantin, Phenytek, Cerebyx)

•Lamotrigine (brand names: Lamictal, Subvenite)

•Phenobarbital (sample brand names: Ѕezаbу, Donnatal)

●Some medicines used to treat infections, especially trimethoprim-sulfamethoxazole ("TMP-SMX"), also called co-trimoxazole (sample brand names: Bactrim, Sulfatrim), as well as other "sulfa" medicines

●Sulfasalazine (brand name: Azulfidine), which is used to treat conditions like rheumatoid arthritis or colitis

●Medicines used to treat pain called "nonsteroidal antiinflammatory drugs" ("NSAIDs"), especially:

•Meloxicam (sample brand name: Mobic)

•Piroxicam (brand name: Feldene)

•Tenoxicam (not available in the US)

Will I need tests? — 

Maybe. Your doctor might be able to tell what is wrong by talking to you and doing an exam. But they might order tests to make sure something else is not causing your symptoms. For example, the doctor might take a sample of skin (called a biopsy) and send it the lab. They might also order a blood test and other tests to check for signs of infection.

How is SJS/TEN treated? — 

Treatment happens in the hospital and usually lasts 2 to 4 weeks. A team of different types of doctors, nurses, and other professionals will help you. Some people with SJS/TEN are treated in the intensive care unit ("ICU") or a burn center.

To treat SJS/TEN, your treatment team will:

●Stop the medicine that caused SJS/TEN (if it was caused by a medicine)

●Try to keep your skin clean and as healthy as possible – This might involve gently removing dead skin, keeping it moist, adding dressings, or doing other things.

●Give you strong medicines to help with skin pain

●Give you a feeding tube so you can get nutrition and fluids

●Check you for signs of infection – This is very important because people with SJS/TEN can develop serious infections.

●Give you antibiotics to treat infection

●Try to keep other affected body parts clean and as healthy as possible – If SJS/TEN affects areas of the pink, moist skin lining your mouth, eyes, vagina, or penis, you will need certain treatments. For example, if your eyes are affected, your doctor might give you saline rinses, "artificial tear" drops or ointments, or antibiotic drops.

●Possibly prescribe a medicine such as cyclosporine (sample brand names: Gengraf, Neoral) – This might help slow down your symptoms.

What happens after I have had SJS/TEN? — 

If you have had SJS/TEN, you must be very careful to never take the medicine that caused it again. You should also avoid any closely related medicines. If you take the same medicine again or one that is closely related, it could kill you. For example, if a "sulfa" antibiotic caused SJS/TEN, you also need to avoid other "sulfa" antibiotics and closely related medicines like sulfasalazine (used to treat colitis or rheumatoid arthritis). Your doctor or nurse should give you a list of medicines you need to avoid taking (table 1).

It's also important to learn all the names of the medicines you should avoid. Medicines often go by more than 1 name, so you might have to learn a few.

You should wear a medical alert bracelet or necklace at all times that lists the medicine or medicines you must avoid and why. If you cannot wear a bracelet or necklace, you should carry a document called an "allergy passport" or "allergy card" at all times.

Make sure your pharmacy makes a note in your record that you had SJS/TEN. Your pharmacy should also have a list of the medicines you must never take.

If you had SJS/TEN caused by allopurinol or an anti-seizure medicine, your doctor might suggest your family members take a blood test. This can show if they are also at risk of getting SJS/TEN with the same medicine. If so, they also need to be careful to avoid that type of medicine.

When should I call the doctor? — 

Call for emergency help (in the US and Canada, call 9-1-1) or go to the emergency department right away if you have:

●Painful patches of skin that are red or purple on your face, chest, back, legs, or arms (picture 1 and picture 2)

●Sores, swelling, and crusting on areas of the pink, moist skin lining your mouth, eyes, vagina, or penis (picture 3 and picture 4)

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