What's SJS?

SJS is a rare, severe skin condition brought on by an adverse reaction to a medication. Countless drugs can precipitate SJS, but the most common medications include non-steroidal anti-inflammatory drugs (e.g. ibuprofen), antibiotics (e.g. penicillins), anticonvulsants (e.g. phenytoin), and allopurinol. SJS can affect anyone taking medications but typically targets those under the age of 30.

 

SJS starts with flu-like symptoms and is followed by a painful rash that spreads over the body, blisters, and peels. SJS and TEN are differentiated by the proportion affecting the entire body. When less than 10% of the entire body is involved, it is recognized as SJS; over 30% is called TEN. Without an early diagnosis, SJS/TEN can lead to systemic bacterial infections, pneumonia, multiple system organ failure and death. The risk of death for SJS is approximately 1-5%, and for TEN 25%-35%.

 

However, survivors of SJS/TEN have life-long complications affecting their eyes, hair, genitourinary tract, and more. While there are no definitive documented statistics on the effects of SJS/TEN within the Canadian population, there are over 50 known cases nationwide, including children, teens, adults and elders.  The cases include both males and females of a variety of races.

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Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are severe skin reactions often triggered by medications or infections. They involve widespread skin detachment and mucous membrane involvement, leading to significant morbidity and mortality.

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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.

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 patients' quality of life

Depending on the stage many patients will die. For the 80% who survive there are life-long medical, physical, and 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, and 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 conditions.

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

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

 

Here is a summary of their long-term effects:

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1. Skin and Mucous Membranes:
Scarring: Severe scarring and pigmentation changes can occur, especially in areas where the skin has detached.
Dryness and Sensitivity: Chronic dryness and sensitivity of the skin can persist, requiring ongoing dermatological care.
Nail Abnormalities: Permanent nail loss or deformities may occur due to damage to the nail beds.

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2. Ocular Complications:
Chronic Dry Eye: Damage to the tear ducts can lead to persistent dry eye syndrome.
Vision Impairment: Scarring of the cornea, conjunctiva, or eyelids can result in vision loss or blindness.
Photophobia: Sensitivity to light may persist due to ocular surface damage.

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3. Respiratory System:
Chronic Respiratory Issues: Damage to the respiratory mucosa can lead to chronic cough, bronchitis, or even pulmonary fibrosis.
Airway Obstruction: Severe cases may lead to long-term airway obstruction requiring medical intervention.

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4. Gastrointestinal Tract:
Esophageal Strictures: Scarring in the esophagus can lead to strictures, causing swallowing difficulties.
Malabsorption: Damage to the gastrointestinal lining may result in long-term absorption issues.

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5. Genital and Urinary Tract:
Strictures and Scarring: Scarring can cause strictures in the urinary tract and genital area, leading to difficulties in urination and sexual dysfunction.

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6. Psychological Impact:
Post-Traumatic Stress Disorder (PTSD): The traumatic experience and prolonged recovery can lead to PTSD, depression, and anxiety.
Body Image Issues: Visible scarring and disfigurement can cause significant distress and impact self-esteem.

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7. Neurological Complications:
Peripheral Neuropathy: Some patients may experience nerve damage leading to chronic pain or sensory issues.

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8. Immune System:
Long-term Immunological Changes: SJS/TEN can lead to changes in immune system function, potentially increasing susceptibility to infections or autoimmune disorders.

These long-term effects necessitate a multidisciplinary approach to care, involving dermatologists, ophthalmologists, pulmonologists, gastroenterologists, urologists, psychologists, and other specialists to manage the diverse and complex sequelae of these conditions. Regular follow-up and comprehensive care plans are essential for improving the quality of life for affected individuals.

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