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

What is Stevens-Johnson syndrome? — Stevens-Johnson syndrome, also called "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 your mouth, nose, eyes, genitals, and other body parts.

A more severe form of SJS is called "toxic epidermal necrolysis" or "TEN." Both conditions are treated in very much the same way.

SJS/TEN is an emergency and requires treatment in a hospital. This is explained more below.

What are the symptoms of SJS/TEN? — The most important symptoms of SJS/TEN are those that affect the skin. In the first few days, before the skin symptoms start, SJS/TEN causes:

●Fever (often higher than 102°F or 39°C)

●Flu-like illness

●Itching or burning eyes

●Joint pain

●Cough

Later, the symptoms of SJS/TEN include:

●Patches of painful, red or purple skin – The patches might look darker in the middle and lighter around the outside.

●Bruises that can have a blister on top

●Blisters followed by peeling of dead skin on the face, chest, back, and limbs

●Sores, swelling, and crusting on the pink, moist skin lining the mouth, eyes, vagina, penis, lungs, and other areas – 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 build-up in the lungs, or lung infections

What causes SJS/TEN? — SJS/TEN is a rare side effect of certain medicines that cannot be predicted. It can also be caused by an infection such as HIV or another viral infection, especially in children.

There are many medicines that can cause SJS/TEN. The chance that one of these medicines will cause SJS is highest during the first 8 to 10 weeks of taking it. 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 names: Aloprim, Zyloprim)

●Some medicines used to treat infections, especially:

•Trimethoprim-sulfamethoxazole, also called co-trimoxazole or TMP-SMX, and other "sulfa" medicines (sample brand names: Bactrim DS, Septra, Sulfatrim)

•Nevirapine (brand name: Viramune), which is an HIV medicine that is not used often in most countries

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

•Carbamazepine (sample brand names: Tegretol, Carbatrol)

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

•Lamotrigine (brand name: Lamictal)

•Phenobarbital (sample brand names: Luminal, Donnatal)

●Medicines used to treat pain called "non-steroidal antiinflammatory drugs," or "NSAIDs," especially:

•Meloxicam (sample brand names: Mobic, Vivlodex)

•Piroxicam (brand name: Feldene)

•Tenoxicam (brand name depends on country)

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

Should I see a doctor or nurse? — Yes, if you develop symptoms of SJS/TEN, go to the emergency department right away or call for an ambulance (in the US and Canada, call 9-1-1).

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. There you will see a team of different types of doctors, nurses, and other professionals. 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 pain in your skin

●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

●Possibly give you other medicines that might help slow down your symptoms – This might include a steroid (such as prednisone) or cyclosporine.

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

You should also 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. For example, if a "sulfa" antibiotic caused SJS/TEN, you also need to avoid closely related medicines like sulfasalazine (used to treat colitis or arthritis) and some antibiotic creams, lotions, and eye drops (such as sulfacetamide drops and silver sulfadiazine cream). Your doctor or nurse should give you a list of medicines you need to avoid taking (table 1).

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 that your family members take a blood test. The test can show if the person is also at risk of getting SJS/TEN with the same medicine. If so, they will also need to be careful to avoid that type of medicine.

More on this topic

Patient education: Side effects from medicines (The Basics)
Patient education: Brand versus generic medicines (The Basics)
Patient education: Sulfa drug allergy (The Basics)
Patient education: Penicillin allergy (The Basics)
Patient education: Medication safety (The Basics)