Research at Vanderbilt University About Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

Vanderbilt University Medical Center

  • Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and serious disorders of the skin across a spectrum of severity usually triggered by an extreme reaction to an infection or medication. The disease presents like a severe burn where the most superficial layer of the skin and top layers in the mouth, genital tract and eyes are affected. This top layer of the skin dies. Since the skin is the most important barrier to invaders this leaves a patient vulnerable to death from severe infection.

  • SJS/TEN affects about 5 million people worldwide each year. It is often detected late, after it has caused serious damage to the skin, eyes and lungs. In many cases, it is fatal. Survivors often feel abandoned and fearful of medications and medical care. Many remain in seclusion for the rest of their lives because of its devastating effects.

  • Elizabeth Phillips, MD, of Vanderbilt University Medical Center, is an internationally renowned expert on SJS/TEN and other severe reactions to drugs. Dr. Phillips and her colleagues have been working to raise awareness of SJS/TEN among health care providers and lay people internationally. They are frequently called upon for advice on diagnosis and treatment of SJS/TEN. They can help give patients advice on drugs safe to take in the future and steer patients and their families away from drug that could represent a risk.
  • The team at Vanderbilt continue to identify genes and risk factors that are associated with SJS/TEN and other serious immune mediated adverse drug reactions. The suspect genes may differ depending on the patient’s ethnic background and the drug that causes the reaction. There is a large number of gene-ethnicity-drug combinations to be investigated. Sometimes the equation is simple and can lead to a test being implemented into routine preventive care but often more research is required to not only prevent SJS/TEN and other serious immune mediated adverse drug reaction but define the mechanisms by which these reactions occur. This is leading to discoveries that it is anticipated will lead not only to prevention when possible but also to earlier diagnosis and better treatments.

  • Because of this diversity, researchers need to obtain saliva samples and medical records from a large number of patients so they can find patterns that will lead to better strategies for prevention and management of SJS. They currently have samples and records from 200 patients and need at least 500.

  • Because the syndrome is rare, they need to obtain samples and records from patients across the United States and internationally through collaborations in Europe, Asia, Africa and South America. It costs money to obtain and analyze the saliva samples and records.

  • Ultimately, the key to better understanding SJS/TEN is to analyze samples from family members of SJS patients who have the same gene but have tolerated the same drug without ill effects. This will help scientists understand other factors such as early life infections and immune events that may contribute to the later development of SJS/TEN.

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