Katie Niemeyer, patient advocate, discusses her experience with Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN).
SJS/TEN is a very severe reaction that causes skin tissue to die and detach. SJS and TEN previously were thought to be separate conditions, but they are now considered part of a disease spectrum. SJS is at the less severe end of the spectrum but all forms of SJS/TEN are a medical emergency that can be life-threatening. The first symptoms of SJS/TEN often include fever and flu-like symptoms. Within about 1 to 3 days, a red or purplish rash forms, and then the skin begins to blister and peel, leading to “raw” areas of skin that are painful. The mucous membranes may also become involved during this time, which can lead to symptoms such as severe conjunctivitis, trouble swallowing and breathing, and difficulty urinating and genital pain.
SJS/TEN often is triggered by certain medications including allopurinol, anti-epileptics, pain relievers, cancer therapies, or antibiotics. SJS/TEN can also be triggered by infections such as pneumonia, herpes virus, and hepatitis A. In many cases the cause cannot be identified. The HLA-B gene has shown an association with higher risk of SJS/TEN, however not everyone with the disease has this gene mutation. Testing for this gene mutation, as well as a skin biopsy test, have made progress in identifying risk before prescribing high-risk medications.
The Power of One
At the 2025 World Orphan Drug Congress, Ms. Niemeyer talked about her story with SJS/TEN. At 16 years old, Ms. Niemeyer had an adverse reaction to an anti-depressant medication that led her to spend three weeks in the hospital with second degree burns on her entire body. During her time in the burn unit, she met a firefighter named Kerry who would stop by her room every day and ask how she was doing and provide words of encouragement. Ms. Niemeyer accredits Kerry’s kindness to her being able to pull through such a difficult time. Now Ms. Niemeyer spends her time as a speaker, discussing the “power of one” and how important empathy and kindness can be. Learn more at https://katieniemeyer.com/
To learn more about SJS and other rare skin conditions, visit https://checkrare.com/diseases/skin-conditions/