Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN): Immunologic Reactions

Zimmerman, Danielle; Dang, Nam Hoang

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are immunologic reactions to several stimuli, mostly medications, which present as a spectrum of primarily widespread mucocutaneous lesions, but also with other organ involvement. Pathology is characterized by full thickness necrosis of the epithelial layer of the involved organ due to immune-mediated apoptosis of the resident keratinocytes. High suspicion for early detection and quick withdrawal of the culprit medication are the most important steps in stopping this reaction. Aggressive supportive care is often necessary as the patient recovers. Steroids, other immunosuppressants, and plasmapheresis have all been studied as treatments, but high-quality evidence supporting their contributions, either together or separately, in decreasing length of hospital stay or prolonging survival have not been consistently demonstrated. Further studies of the mechanism of action and novel treatment modalities are still needed to improve outcomes in patients with this rare but often fatal condition.