In 1922, S.C. Johnson discovered a “new disease” while treating a child with disturbing symptoms. What the doctor dubbed “Stevens-Johnson Syndrome” (SJS) is a severe skin reaction that proves fatal for many patients. (Advanced SJS cases are also commonly known as toxic epidermal necrolysis, or TEN.) This life-threatening skin condition causes cell death, which separates the epidermis layer from the dermis. Seemingly “harmless” over-the-counter drugs, such as Advil, Tylenol, and Motrin, trigger most diagnosed SJS cases. While the condition is rare, suspected cases should seek medical treatment immediately. In fact, just over 50% of untreated SJS patients die. We’ll explain more about this little-known skin condition below in our Stevens-Johnson Syndrome/toxic epidermal necrolysis overview.

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: Uncovering the Disease’s History

Physicians Albert Stevens and Frank Johnson consulted on a case involving two young boys in 1922. Both children were admitted to the hospital with dark red and purplish spots, fever, conjunctivitis and total loss of vision.

The physicians, who discovered what would become Stevens-Johnson Syndrome/toxic epidermal necrolysis, asked multiple consultants to view the boys and their condition. That report contained the first reference to “Stevens-Johnson Syndrome,” now categorized as a severe mucocutaneous disease.

In 1956, Dr. Alan Lyell described four patients that had topical eruptions “resembling scalding of the skin.” Shortly afterwards, Dr. Lyell called this eruption toxic epidermal necrolysis, or TEN. Upon further review, it became clear that TEN was an advanced drug-induced reaction. Stevens-Johnson Syndrome/toxic epidermal necrolysis comprise the most extreme results from a severe epidermolytic drug reaction. The only measurable difference between these conditions is the extent to which the patient’s skin peels off.

What Drugs Can Trigger Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN)?

Many over-the-counter (OTC) and prescription drugs can trigger allergic reactions that, if left untreated, become Stevens-Johnson Syndrome/toxic epidermal necrolysis. Allopurinol, a uric acid reducer that treats gout and kidney stones, is the most commonly cited culprit. However, children may also develop the condition after taking pain or fever-reducing medications.

Here are just a few prescription and OTC medications linked to Stevens-Johnson Syndrome/toxic epidermal necrolysis:

• Antibiotics (penicillin, Zithromax, Cipro, Flagyl)
• Sulfa drugs (Bactrim, Septra)
• NSAIDs (acetaminophen, ibuprofen, Tylenol, Children’s Motrin, naproxen sodium)
• Anticonvulsants (Dilantin/phenytoin, Carbamazepine, Tegretol, Onfi)
• Anti-epileptics (Lamictal/lamotrigine)
• Other medications used for arthritis, gout, infections, seizure, pain relief or mental illness symptoms

What Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Symptoms Look Like

Due to the severity of Stevens-Johnson Syndrome/toxic epidermal necrolysis, those presenting with symptoms should seek medical attention immediately. Rapid diagnosis as well as trigger drug cessation are crucial for the patient’s survival. Once diagnosed, patients are typically transferred into intensive care or a burn unit for treatment. Due to high mortality rates (averaging 1-5%), seek help immediately for any rashes that develop after starting a new medication.

The most common initial SJS signs and symptoms include:

• Hives or rash that spreads across the skin
• Blisters on mucous membranes, including mouth, nose, eyes or genitals
• Flu-like symptoms (fever, cough, chills)
• Skin peeling off (10% or less)
• Swollen, red eyes/eyelids
• Conjunctivitis

Toxic epidermal necrolysis is even more dangerous than SJS, with an estimated 25%-35% mortality rate. However, for elderly patients, the TEN mortality rate is even higher. More than half of all TEN survivors experience long-term complications and side effects. Commonly reported TEN symptoms include:

• Total skin loss of 30% or more
• Loss of nails
• Cellulitis
• Sepsis
• Organ failure
• Blindness
• Internal organ damage (lungs, heart, liver, kidneys)
• Permanent skin damage
• Death

How Common Is Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN)?

Both SJS and TEN are rare but potentially fatal skin conditions that affect approximately 1-2 people per million each year. Their appearance almost appears random, affecting nearly anyone in the population — including infants as well as young children. However, certain infectious diseases may impact TEN incidence rates.

According to the Mayo Clinic, the following infections increase your chances for developing either SJS or TEN:

• Herpes virus (simplex or zoster)
• Pneumonia
• HIV
• Hepatitis A

In addition, some infectious diseases have a pronounced impact on SJS/TEN incidence rates. For example, those with HIV have approximately 100-fold higher risk for developing SJS/TEN than the general population.

Other SJS/TEN risk factors include:

• A weakened immune system
• Prior SJS/TEN diagnosis
• A family history of SJS/TEN
• The HLA-B*1502 gene (primarily affects families of Chinese, Southeast Asian or Indian descent)

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Lawsuit Information

Because allergic reactions that evolve into full-blown SJS/TEN are rare, many pharmaceutical companies fail to warn consumers about the risk. As a result, many patients (including parents and children) don’t know that prescription or OTC medications can cause SJS/TEN symptoms. Warning labels rarely list SJS/TEN risks, leading deceased victims’ families to file wrongful death lawsuits against drug manufacturers. Patients who recover from SJS/TEN outbreaks may also seek compensation and justice through medical malpractice or failure to warn claims.