Stevens-Johnson syndrome, also known as SJS or toxic epidermal necrolysis (TEN), is a rare but serious disorder of the skin and mucous membranes. It is caused by a genetic predisposition to have an adverse reaction to certain medications.
Stevens-Johnson Syndrome: An Overview
The amount of skin involvement determines whether the disease is called SJS or TEN. If more than 30% of the total body surface area is involved, it is called toxic epidermal necrolysis or the acronym TEN.
SJS/TEN is a very serious condition that causes blistering and sloughing of the skin and mucous membranes. The effect is similar to a severe full-thickness burn. The body becomes at risk for fluid loss, infection, organ failure, blindness, and death.
Get in TouchSymptoms of Stevens-Johnson Syndrome
The initial symptoms of SJS and TEN can include rash, fever, sore throat and mouth, fatigue, and burning eyes. As the disease progresses, symptoms can include:
- Widespread skin pain
- Red or purple rash spreading over the body
- Blisters on the skin, mouth, and mucous membranes of the eyes and genitals
- Shedding of the skin (“erosions”) once blisters form, leaving raw skin
Other symptoms may include drooling, swollen eyelids, painful urination, and peeling skin. Secondary symptoms can include infection in areas where the skin has peeled away, leaving exposed flesh.
Get in TouchCauses of Stevens-Johnson Syndrome
Most SJS cases are due to allergic reactions to medication. TEN is considered by many medical experts to be exclusively caused by pharmaceutical drugs. Changes in a gene called HLA-B seem to make the body more likely to attack the skin cells when an allergic reaction is triggered.
Genetic factors, such as alleles, may be responsible for the few cases of SJS which have no apparent cause.
Adverse Drug Reactions
The drugs most associated with SJS include:
- Anti-gout medications
- Anti-convulsants and anti-psychotics
- Sulfonamides
- Non-steroidal anti-inflammatories (NSAIDs)
- Antibiotics
Infections
Some of the most high-risk infectious conditions for developing SJS/TEN are:
- HIV
- Systemic lupus erythematosus
- Cancer
- Other weakened immune system or connective tissue diseases
Treatment Options for SJS
Most treatment for SJS/TEN is supportive. The first step is to stop using whatever medication is causing the adverse reaction. Once the drug has been removed, it still must clear from the patient’s system, which may take time, depending on how much was ingested before symptoms appeared.
Supportive care can include:
- IV fluid replacement to prevent electrolyte imbalance.
- High-calorie food — by nasogastric tube if necessary — to increase energy and promote healing.
- Use of non-adhesive dressings on blisters and exposed skin.
- IV immunoglobulins if there is extensive fluid or blood loss.
- Skin grafts or membrane grafts (for the eyes) if needed.
- Antibiotics or cyclosporine for infections.
- IV steroids to prevent swelling of affected mucous membranes.
- Treatment in a burn unit or intensive care unit if too much body surface is involved.
In cases involving the mouth or nasal tissue, oxygen therapy may be necessary to support the patient’s breathing. Complications of SJS/TEN can include pneumonia, sepsis, and death in up to 30%-80% of TEN cases.
If you need legal advice regarding SJS, contact Dunn Sheehan LLP at 214.462.7582 or make an appointment for a consultation today.
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