Stevens-Johnson Syndrome

Stevens-Johnson Syndrome

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 to 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.
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 show up, 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 red and painful skin – The patches might look darker in the middle and lighter around the outside
Bruises that can have a blister on top
Blisters or peeling skin on the chest, face, palms of hands, or soles of feet
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 a medicine that cannot be predicted. But it can also happen to people who have an infection, such as HIV. SJS/TEN can sometimes happen in children who have certain infections, too.
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 (brand names: Aloprim, Zyloprim, Duzallo)
Some medicines used to treat infections, especially:
•Trimethoprim-sulfamethoxazole, also called co-trimoxazole or TMP-SMX, and other "sulfa" drugs (sample brand names: Bactrim DS, Septra, Sulfatrim, Pediazole)
•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-epileptics," including:
•Carbamazepine (sample brand names: Tegretol, Carbatrol)
•Phenytoin and fosphenytoin (brand names: Dilantin, Phenytek, Cerebyx)
•Lamotrigine (brand name: Lamictal)
•Phenobarbital (brand names: Luminal, Donnatol)
Medicines used to treat pain called "non-steroidal antiinflammatory agents," or "NSAIDs," especially:
•Meloxicam (sample brand name: Mobic, Vivlodex)
•Piroxicam (brand name: Feldene)
•Tenoxicam (brand name depends on country)
Should I see a doctor or nurse? — Yes, if you develop symptoms of SJS/TEN, go to the emergency room right away or call for an ambulance (in the US and Canada, dial 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 he or she might order tests to make sure something else is not causing your symptoms. For instance, the doctor might take a sample of skin (called a biopsy) and send it the lab. He or she 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
Give you a medicine called cyclosporine, which might help slow down your symptoms
Possibly give you medicines to reduce inflammation, such as prednisone.
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 or 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" at all times.
You should also learn all the names of the medicines you should avoid. Medicines often go by more than one 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 drugs 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-epileptic 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.
All topics are updated as new evidence becomes available and our peer review process is complete.
This topic retrieved from UpToDate on: Mar 30, 2020.
Topic 87114 Version 11.0
Release: 28.2.2 - C28.105
© 2020 UpToDate, Inc. and/or its affiliates. All rights reserved.