Exfoliative Skin Diseases: Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Stevens-Johnson Syndrome (SJS), Toxic Epidermal Necrolysis (TEN), and SJS/TEN overlap syndrome represent a spectrum of rare, but life-threatening type IV hypersensitive reactions to medications or infections. The clinical diagnosis of SJS versus SJS/TEN overlap, versus TEN is based upon the severity or amount of skin desquamation. The loss of skin increases the risk of infection, sepsis, multiple organ dysfunction, and death. Mortality is related to the amount of skin desquamation, patient risk factors, and comorbidities. Management is primarily supportive care, but specialized centers often use immunomodulating therapies to attempt to reduce the severity of the hypersensitivity reaction. Most important treatment options are to stop the causative agent, resuscitate, and promptly transfer to a specialized center able to treat SJS/TEN with multi-disciplinary specialists that can effectively treat the acute syndrome and mitigate long-term consequences.