What is Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)

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• SJS and TEN represent different degrees of severe, acute, life-threatening mucocutaneous reactions mainly resulting from drugs.

• SJS/TEN causes the skin to become red and purple, tender, and peel away from the body. These diseases affect not only the skin on the external surface of the body but also the moist lining of the mouth, nose, eyes, genitals, and other body parts.

• Both conditions are considered a medical emergency that requires hospitalization and patient management is usually provided in intensive care units or burn centers.

• Recovery can take weeks to months, and there are numerous long-term sequelae.

• Medications are the major precipitating cause and some drugs have a greater genetic risk factor. Newer treatments include safe anti-inflammatory biological injections like Etanercept.

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WOMEN

are nearly 1.5x more likely to experience SJS/TEN than men.

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SJS/TEN AFFECTS

1-7 cases per million patients.

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Additional Information about SJS / TEN:

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Definition of syndrome

A life threatening disease with blistering of the skin and eroding of mucous membranes (eyes, mouth, genitals).

 

Cause

80% are caused by an identifiable drug, 20% are unknown. Drugs are varied but anti-seizure medications, sulfonamides and allopurinol are top of the list.

 

Qualifiers of the syndrome

The severity of SJS to TEN depends on the amount of skin involved (can be 100%) and all patients with SJS/TEN will need to be treated in an Intensive Care. 

• The term SJS is used when the blistering of the skin involves less than 10% of the total body surface area.

• The term TEN is used when the blistering involves more than 30% of the body surface area. 

• The term SJS/TEN overlap is used when there is blistering that covers between 10 to 30% of the body surface area.

 

Impact on the patients' quality of life

Depending on the stage many patients will die. For the 80% who survive there are life-long medical, physical, psychological traumas that persist.

 

Treatment

Early treatment is life-saving.  Different stages might need different treatments.  Intensive Care Burn Centers are optimal.

Therapies include immune modulators like corticosteroids, anti-TNF (e.g., Etanercept) biologicals, amnion for eye involvement.

 

Prevalence rates in Canada

Perhaps 1 to 2 per million Canadians will be afflicted per year.  

 

Some Facts​

• The condition is rare so it might be missed. 

• Delay is a risk for a poor outcome. 

• There are genetic tests for some patients that might help reduce the risk.

• Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis are two rare, acute, but life-threatening skin condition.

• Both conditions are usually an allergic reaction to medication, however; in some cases, the causes remain unknown.

• Approximately 1-7 people per million are affected globally each year

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