The Signs and Symptoms of Stevens-Johnson Syndrome

As a rare dermatologic condition, Stevens-Johnson syndrome (SJS) may not be at the forefront of thought among diagnosing clinicians when observing finely discernible signs and symptoms. But specialists have methodology—and ideal roles—designed to identify and address the potentially fatal disease.

In an interview with HCPLive during the Society of Dermatology Physician Assistants (SDPA) 2022 Annual Meeting, Travis Vandergriff, MD, associate professor in the department of dermatology at UT Southwestern Medical Center, discussed the manifestation, presentation and diagnosis of SJS.

The very rare disease, with an incidence of approximately 1 in 1 million persons, represents a “severe cutaneous drug reaction” generally caused by an allergic response. The most common triggers include anti-epileptic, antibiotics, alpurinol, about 7-10 days after initiated care.

“It develops over time, usually within a week and often shorter than that, into a blistering eruption and affects the mucus membranes,” Vandergriff said. “When the skin blisters, that puts patients at risk of infection, so there is some risk of mortality.”

Some ethnic and race subpopulations are at increased risk of developing SJS based on medications, and identifiable risk factors exists. ““But for the most part it’s rare and difficult to predict who would be potentially susceptible to it,” Vandergriff explained. “It is something that, because it’s such a severe and life-threatening eruption, even trough it’s rare, it’s helpful to be aware of it.”

In the event of SJS presentation, Vandergriff believes dermatologists play the most critical role in early diagnosis: what may look non-specific to a primary care or other frontline caregiver may be distinct to them. He noted a dermatologist may be able to pick up on “subtle clues” including symptoms of skin pain, early blistering, a dusky rash appearance.

And while the disease management strategy can entail a variety of specialists, diagnosis is on the dermatologist in the most ideal scenario.

“So you want to make sure you’re taking a good history, and for patients on high-risk medications, the diagnosis should cross your mind even if there’s no blistering,” Vandergriff advised.