Stevens-Johnson Syndrome

What is Stevens-Johnson syndrome (SJS)?

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious skin conditions that cause your skin to develop rashes, blisters, and then peel. Your mucus membranes, including your eyes, genitalia and mouth, are also affected. If you get this condition, you’ll likely be admitted to a hospital.

Some people consider SJS and TEN to be different diseases, while others consider them to be the same disease but at different levels of severity. SJS is less intense than TEN. (For instance, skin peeling might affect less than 10% of the entire body in SJS, while skin peeling affects more than 30% of the body in TEN.) However, both conditions can be life-threatening.

Are there other names for Stevens-Johnson syndrome (SJS)?

Yes. It is also known as Lyell’s syndrome, Stevens-Johnson syndrome/toxic epidermal necrolysis, and Stevens-Johnson syndrome toxic epidermal necrolysis spectrum. It might be called drug-induced Stevens-Johnson syndrome or mycoplasma-induced Stevens-Johnson syndrome if it’s linked to a specific cause.

Who gets Stevens-Johnson syndrome (SJS)?

Many cases of SJS happen in children and adults younger than 30 years old, but also occur in others, especially the elderly. More cases of SJS occur in females than males. Infections, like pneumonia, are the most likely cause of SJS in children, whereas medications are the most likely cause of SJS/TEN in adults.

What are the risk factors for developing Stevens-Johnson syndrome?

A combination of factors is likely involved in developing these disorders, including a genetic bias. Environmental factors might cause the gene to be triggered. One of these genetic factors include specific human leukocyte antigens (HLAs) that may increase one’s risk of developing SJS or TEN.

What are the symptoms of Stevens-Johnson syndrome (SJS)?

Symptoms of Stevens-Johnson syndrome include:

• Skin pain.
• Fever.
• Body aches.
• Red rash or red blotches on your skin.
• Cough.
• Blisters and sores on the skin and on mucus membranes of the mouth, throat, eyes, genitals and anus.
• Peeling skin.
• Drooling (because closing the mouth is painful).
• Eyes sealed shut (due to blisters and swelling).
• Painful urination (due to blistered mucus membranes).

What causes Stevens-Johnson syndrome (SJS)?

Causes of Stevens-Johnson syndrome include:

• Allergic reaction to a medication (most cases of SJS and almost all cases of TEN).
• Infections, like mycoplasma pneumonia, herpes and hepatitis A.
• Vaccinations.
• Graft-versus-host disease.
• No known cause.

If your case of SJS is caused by a drug, symptoms appear about one to three weeks after you start taking medication. The flu-like illness (fever, cough and headache, skin pain) is followed first by a rash and then peeling. In the case of TEN, some people even lose hair and nails.

What drugs are most likely to cause Stevens-Johnson syndrome?

Medications most likely to cause Stevens-Johnson syndrome include:

• Antibacterial sulfa drugs.
• Anti-epileptic drugs, including phenytoin (Dilantin®), carbamazepine (Tegretol®), lamotrigine (Lamictal®), and phenobarbital (Luminal®).
• Allopurinol (Aloprim®, Zyloprim®), a drug used to treat gout and kidney stones.
• Non-steroidal anti-inflammatory drugs (NSAIDs), including piroxicam (Feldene®), nevirapine (Viramune®), and diclofenac (Cambia®, Flector®).
• Antibiotics.

Are there other factors that increase the risk of someone developing Stevens-Johnson syndrome (SJS)?

You are at greater risk of SJS if you have the following conditions:

• Bone marrow transplant.
• Systemic lupus erythematosus.
• Human immunodeficiency virus (HIV).
• Other chronic diseases of joints and connective tissue.
• Cancer.
• Weakened immune system.
• Family history of SJS.
• Variation of a specific gene called human leukocyte antigen-B.

How is Stevens-Johnson syndrome (SJS) diagnosed?

Healthcare providers diagnose SJS and TEN:

• By looking at the skin and mucous membranes affected (typically at least two mucous membranes are affected).
• By your pain level.
• By how fast your skin has been affected.
• By how much of your skin has been affected.
• By taking a skin biopsy.

How is Stevens-Johnson syndrome treated?

Treatments for Stevens-Johnson syndrome include:

• Stopping the medication that has caused the problem.
• Replacing electrolytes with intravenous (IV) fluids.
• Using non-adhesive dressings on the affected skin.
• Using high-calorie food, possibly by tube-feeding, to promote healing.
• Using antibiotics when needed to prevent infection.
• Providing pain relief medications.
• Treating you in a hospital, possibly even in an intensive care or burn unit.
• Using specialist teams from dermatology and ophthalmology (if your eyes are affected).
• In some cases, treating you with IV immunoglobulin, cyclosporine, IV steroids, or amniotic membrane grafts (for your eyes).

What are the complications of Stevens-Johnson syndrome (SJS)?

The most severe complication of SJS and TEN is death. Death happens in about 10% of cases of SJS, and about 50% of TEN. Other complications could include:

• Pneumonia.
• Sepsis (massive bacterial infections).
• Shock.
• Multiple organ failure.

Can Stevens-Johnson syndrome be prevented?

No. Since Stevens-Johnson syndrome is, in most cases, triggered by medications, there’s no way to know – before taking medications – that you might experience an adverse reaction to the drug. If a medication is identified that has triggered this condition, you will want to avoid that drug or related drug again.

What is the outlook for people who have Stevens-Johnson syndrome?

Each person’s experience with Stevens-Johnson syndrome can be different. Skin can regrow in a matter of weeks, but recovery can take months if symptoms are severe. Some long-term reactions may develop, including:

• Skin: dryness, itching, change in skin color.
• Eyes: chronic swelling and/or dryness, chronic irritation, difficulty seeing, sensitivity to light (photophobia).
• Excessive sweating.
• Lung damage, chronic obstructive pulmonary disease, asthma.
• Nail loss or deformity.
• Hair loss (alopecia).
• Dryness of mucus membranes, which could cause problems urinating.
• Chronic fatigue syndrome.
• Difficulties with your sense of taste.

SJS may redevelop if you are exposed to the same medication known to have triggered the condition the first time. In such cases, the second episode is usually more severe than the first episode.

SJS results in death in 10% of patients and in 30% for those with TEN – due mostly to sepsis, acute respiratory distress syndrome and multiple organ failure.