Kids' Health Stevens-Johnson Syndrome and Its Causes, Symptoms, and Triggers By Vincent Iannelli, MD Updated on March 19, 2024 Medically reviewed by Lyndsey Garbi, MD Print Table of Contents View All Table of Contents What It Is Symptoms Causes Treatment Stevens-Johnson syndrome (SJS) is a rare condition of the skin and mucous membranes. It is usually thought of as a severe reaction to a medication or infection, though some experts think it is a separate condition. There is also a severe form of Stevens-Johnson syndrome called toxic epidermal necrolysis (TEN) or Lyell's Syndrome. This article discusses Stevens-Johnson syndrome and its causes. It also goes over the symptoms and triggers of this condition. HRAUN / Getty Images What Is Stevens-Johnson Syndrome? Stevens-Johnson syndrome can be life-threatening and can cause serious symptoms, such as large skin blisters and shedding of a child's skin. Two pediatricians, Albert Mason Stevens and Frank Chambliss Johnson, discovered Stevens-Johnson syndrome in 1922. Children of any age and adults can be affected by Stevens-Johnson syndrome, although people who are immunocompromised, such as those with HIV, are likely more at risk. Stevens-Johnson syndrome is usually thought of as a severe form of erythema multiforme, which is itself a type of hypersensitivity reaction to a medication or infection. This can occur with prescription medications and with (including over-the-counter drugs) or infections like herpes or walking pneumonia caused by Mycoplasma pneumoniae. Other experts think Stevens-Johnson syndrome is a separate condition from erythema multiforme. These experts acknowledge two forms of erythema multiforme: erythema multiforme minor and erythema multiforme major (Stevens-Johnson syndrome). What is the survival rate for Stevens-Johnson syndrome? Unfortunately, about 5.4% of people with Stevens-Johnson syndrome and 15.3% with toxic epidermal necrolysis have such severe symptoms that they do not recover. Symptoms of Stevens-Johnson Syndrome Stevens-Johnson syndrome generally starts with flu-like symptoms, such as a fever, sore throat, and cough. Next, within a few days, a child with Stevens-Johnson syndrome will develop: A burning sensation on the lips, inside of their cheeks (buccal mucosa), and eyesA flat red rash, which may have dark centers, or develop into blistersSwelling of the face, eyelids, and/or tongueRed, bloodshot eyesSensitivity to light (photophobia)Painful ulcers or erosions in the mouth, nose, eyes, and genital mucosa, which can lead to crusting Complications of Stevens-Johnson syndrome can include corneal ulceration and blindness, pneumonitis, myocarditis, hepatitis, hematuria, kidney failure, and sepsis. A positive Nikolsky's sign, in which the top layers of a child's skin comes off when rubbed, is a sign of severe Stevens-Johnson syndrome or a sign that it has evolved into toxic epidermal necrolysis. A child is also classified as having toxic epidermal necrolysis if they have more than 30% epidermal (skin) detachment. What Is Most Likely to Cause Stevens-Johnson Syndrome? Although more than 200 medications can trigger Stevens-Johnson syndrome, the most common include: Anticonvulsants (epilepsy or seizure treatments), including Tegretol (Carbamazepine), Dilantin (Phenytoin), Phenobarbital, Depakote (Valproic Acid), and Lamictal (Lamotrigine) Sulfonamide antibiotics, such as Bactrim (Trimethoprim/Sulfamethoxazole), which is often used to treat UTIs and MRSA Beta-lactam antibiotics, including penicillins and cephalosporins Nonsteroidal anti-inflammatory drugs, especially of the oxicam type, such as Feldene (Piroxicam) (not usually prescribed to children) Zyloprim (allopurinol), which is typically used to treat gout Stevens-Johnson syndrome is usually thought to be caused by drug reactions, but infections can also trigger it, including those caused by: Herpes simplex virus Mycoplasma pneumoniae bacteria (walking pneumonia) Hepatitis C Histoplasma capsulatum fungus (Histoplasmosis) Epstein-Barr virus (mono) Adenovirus Cam Stevens-Johnson Syndrome Be Cured? The treatments for Stevens-Johnson syndrome typically begin by stopping whatever drug may have triggered the reaction. The patient will also receive supportive care until they recover, which usually takes about four weeks. These patients often need to be placed in an intensive care unit. Treatments might include: IV fluidsNutritional supplementsAntibiotics to treat secondary infectionsPain medicationsWound careSteroids and intravenous immunoglobulin (IVIG), although their use is still controversial Stevens-Johnson syndrome treatments are often coordinated in a team approach, with the ICU doctor, a dermatologist, an ophthalmologist, a pulmonologist, and a gastroenterologist. Parents should seek medical attention immediately if they think their child might have Stevens-Johnson syndrome. Summary Stevens-Johnson syndrome is a severe reaction to a medication or antibiotic that affects the skin and mucous membranes. It causes serious symptoms like large blisters and skin loss. Stevens-Johnson syndrome can be triggered by medications, including certain antibiotics, anticonvulsants, and prescription NSAIDs. Infections with certain viruses or bacteria can also trigger it. Stevens-Johnson syndrome is usually treated in an intensive care unit with IV fluids, pain medication, wound care, and other supportive treatments. 8 Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. Callahan SW, Oza VS. Stevens-Johnson syndrome-A look back. JAMA Dermatol. 2017;153(2):240. doi:10.1001/jamadermatol.2016.0180 Sadek M, Iqbal O, Siddiqui F, et al. The role of IL-13, IL-15 and granulysin in the pathogenesis of Stevens-Johnson syndrome/toxic epidermal necrolysis. Clin Appl Thromb Hemost. 2021;27:1076029620950831. doi:10.1177/1076029620950831 Sweileh WM. Bibliometric analysis of literature on toxic epidermal necrolysis and Stevens-Johnson syndrome: 1940 - 2015. Orphanet J Rare Dis. 2017;12(1):14. doi:10.1186/s13023-017-0566-8 Wasuwanich P, So JM, Chakrala TS, Chen J, Motaparthi K. Epidemiology of Stevens-Johnson syndrome and toxic epidermal necrolysis in the United States and factors predictive of outcome. JAAD Int. 2023;13:17-25. doi:10.1016/j.jdin.2023.06.014 National Health Service. Steven Johnsons syndrome. Neill BC, Seger EW, Ferguson JE, Hooton T, Rickstrew JJ, Rajpara A. SJS/TENN: A mnemonic for early clinical diagnosis of Stevens-Johnson syndrome and toxic epidermal necrolysis. Kans J Med. 2021;14:114-115. doi:10.17161/kjm.vol1414842 National Institutes for Health. Stevens Johnson syndrome/toxic epidermal necrolysis. Schneider JA, Cohen PR. Stevens-Johnson syndrome and toxic epidermal necrolysis: A concise review with a comprehensive summary of therapeutic interventions emphasizing supportive measures. Adv Ther. 2017;34(6):1235-1244. doi:10.1007/s12325-017-0530-y By Vincent Iannelli, MD Vincent Iannelli, MD, is a board-certified pediatrician and fellow of the American Academy of Pediatrics. Dr. Iannelli has cared for children for more than 20 years. See Our Editorial Process Meet Our Medical Expert Board Share Feedback Was this page helpful? Thanks for your feedback! What is your feedback? Other Helpful Report an Error Submit