Stevens-Johnson syndrome (SJS) is a rare skin reaction. The cause is usually a new medication.
SJS often begins with flu-like symptoms, such as fever, chills, muscle aches, and fatigue. Then a rash appears, which turns into blisters on the skin. The blisters lead to extensive skin peeling that can put you at risk of dehydration and infection.
SJS needs emergency medical treatment. Treatment focuses on controlling the symptoms, preventing complications, and addressing the underlying cause.
Stevens-Johnson syndrome (SJS) is a rare but serious skin condition. The most common cause is an adverse reaction to a medication. SJS causes the skin to get inflamed, blister, and peel. The blistering covers the whole body.
Even though the skin symptoms are most noticeable, SJS can also cause dangerous inflammation in other organs. And the blistering puts people at risk for serious complications, like dehydration and infection. So SJS is a medical emergency that needs prompt treatment to prevent long-term harm.
In this article, we explain:
Causes of SJS, including the most common medications that cause it
Early warning signs
Potential complications and progression
Treatment and diagnosis for SJS
The most common cause of SJS is an adverse reaction to a medication that someone has taken.
More than 100 drugs can cause SJS. Most often these include:
Allopurinol: a medication that treats gout and kidney stones
Here’s a more complete list of the different medications that have been connected to cases of SJS.
A less common cause of SJS is an actual infection. Examples of infections that can do this are:
Mycoplasma pneumonia: Another name for this pneumonia is “walking pneumonia,” because it doesn’t cause severe symptoms. It’s more commonly associated with SJS in children and teens.
Cytomegalovirus (CMV): This common virus can affect people of any age. It generally doesn’t cause any symptoms unless a baby is born with it.
Human immunodeficiency virus (HIV): This virus attacks the body’s immune system.
Herpes simplex virus (HSV): This virus often causes sores on the mouth or genitals.
Epstein-Barr virus (EBV): This virus commonly spreads through saliva. It often leads to infectious mononucleosis, or the “kissing disease.”
Influenza virus: This common infection affects the nose, throat, and lungs.
Coxsackievirus: This virus causes a rash in children, often on their hands, feet, and in their mouth, hence its popular name “hand, foot, and mouth” disease. In adults, it can cause symptoms like the common cold.
SJS might occur for other reasons, too. There have been a few cases of people developing SJS after receiving a vaccination. But these reports are rare, and it isn’t clear if the vaccine itself led to SJS.
Other rare causes of SJS include graft-versus-host disease, a condition that can develop after a bone marrow or organ transplant. And sometimes SJS develops for no known reason.
SJS typically develops within 1 to 3 weeks after starting a medication. It can also start after someone has stopped taking a certain medication (usually within 2 weeks).
It starts with flu-like symptoms, such as:
Stinging sensation in the eyes
These are the earliest signs of SJS. In this stage, most people aren’t aware that their symptoms are from SJS. This is especially true because the symptoms are similar to what you may feel at the start of a cold or the flu.
The rash often starts 1 to 3 days after the other symptoms. And as the rash evolves, it can change in appearance and location from day to day. Here’s the common progression of the SJS rash:
It typically first appears on the upper chest, face, and the hands and feet.
When it first starts, the rash looks like red spots that are darker in the middle and lighter around the edges. These are sometimes called “target” lesions because they look like a bullseye.
Within hours to days, it starts to spread to other parts of the body. Most often it spreads to the abdomen, arms, and legs.
As it spreads, the red spots join together and form fluid-filled blisters.
The blisters eventually burst and the skin begins to peel. This exposes large areas of skin and creates a risk of infection.
The rash is often itchy. And it can also be very painful, especially when the skin starts to blister and heal. This can lead to several other complications:
The exposed skin can easily become infected.
The blisters can develop along the lining of the mouth, making it hard to eat and drink.
The rash can spread to the tubes that carry air to the lungs, making it hard to breathe.
The lining of the genitals and the skin on the penis and vagina can blister, making it painful to urinate and defecate.
Often the eyes and eyelids become red and painful. The eyelids can swell and crust, making it feel like your eyes have sand in them. Some people also experience sensitivity to light.
A healthcare provider can often diagnose SJS just by looking at your skin. The appearance of the rash and the way it spreads is very specific to SJS.
To confirm the diagnosis, a provider can do a skin biopsy. They’ll take a sample of skin and look at it under a microscope.
SJS can be life-threatening when the blistering leads to some of the complications we discussed above. So early recognition and treatment are important.
Treatment for SJS is best in the hospital because SJS creates a risk of dehydration and infection. Severe cases may need treatment in a burn unit because they are equipped to handle skin peeling and wound care.
Since an adverse reaction to a medication causes most cases of SJS, the most important first step in treating SJS is stopping that medication.
A key part of treatment for SJS is supportive and specialized medical care. This includes:
Intravenous fluids to maintain hydration
Daily wound care with nonadhesive dressings
Pain relief medications
High-calorie food to promote healing
Specialized eye care if SJS affects the eyes or eyelids
Antibiotics in the case of skin infection
Some cases need additional treatments. The decision depends on the severity of SJS and if the underlying cause is unknown. These treatments may include:
Cyclosporine: may decrease how long SJS lasts
Plasmapheresis: can help remove parts of the harmful drug from the blood
Tumor necrosis factor (TNF)-alpha inhibitors: can decrease inflammation
Steroids: may improve outcomes if started early
Intravenous immunoglobulin (IVIG): can block the drug from harming the body
And most importantly, if a medication is the likely cause of someone’s SJS, that person needs to avoid using that medication for the rest of their life.
Stevens-Johnson syndrome is a well-known condition due to how scary it can be. The blistering is quite extensive, and few skin conditions can lead to such life-threatening complications. But keep in mind that it’s rare. And with early detection and good wound care, people can make a full recovery. It’s important to be aware of the risks of any medication before you start taking it, but the likelihood you will develop SJS from one of them is low.
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