Kids' Health Stevens-Johnson Syndrome Overview By Vincent Iannelli, MD Vincent Iannelli, MD Facebook 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. Learn about our editorial process Updated on February 10, 2020 Medically reviewed by Lyndsey Garbi, MD Medically reviewed by Lyndsey Garbi, MD LinkedIn Lyndsey Garbi, MD, is a pediatrician who is double board-certified in pediatrics and neonatology. Learn about our Medical Expert Board Print Stevens-Johnson syndrome (SJS) is usually thought of as a severe form of erythema multiforme, which is itself a type of hypersensitivity reaction to a medication, including over-the-counter drugs, or an infection, like herpes or walking pneumonia that is caused by Mycoplasma pneumoniae. Other experts think of Stevens-Johnson syndrome as a separate condition from erythema multiforme, which they instead divide into erythema multiforme minor and erythema multiforme major forms. To make things even more confusing, there is also a severe form of Stevens-Johnson syndrome: toxic epidermal necrolysis (TEN), which is also known as Lyell's Syndrome. HRAUN / Getty Images Stevens-Johnson Syndrome Two pediatricians, Albert Mason Stevens and Frank Chambliss Johnson, discovered Stevens-Johnson syndrome in 1922. Stevens-Johnson syndrome can be life-threatening and can cause serious symptoms, such as large skin blisters and shedding of a child's skin. Unfortunately, about 10% of people with Stevens-Johnson syndrome and 40%-50% with toxic epidermal necrolysis have such severe symptoms that they do not recover. Children of any age and adults can be affected by Stevens-Johnson syndrome, although people who are immunocompromised, such as having HIV, are likely more at risk. Symptoms 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 eyes.A flat red rash, which may have dark centers, or develop into blisters.Swelling of the face, eyelids, and/or tongue.Red, bloodshot eyes.Sensitivity 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 that it has evolved into toxic epidermal necrolysis. A child is also classified as having toxic epidermal necrolysis if they have more than 30% of epidermal (skin) detachment. Causes Although more than 200 medications can cause or 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 that may also be associated with it can include those caused by: Herpes simplex virus Mycoplasma pneumoniae bacteria (walking pneumonia) Hepatitis C Histoplasma capsulatum fungus (Histoplasmosis) Epstein-Barr virus (mono) Adenovirus Treatments The treatments for Stevens-Johnson syndrome typically begin by stopping whatever drug may have triggered the reaction and then supportive care until the patient recovers in about four weeks. These patients often require care in an intensive care unit, with treatments that 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 that their child might have Stevens-Johnson syndrome. 6 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 Harr T, French LE. Toxic epidermal necrolysis and Stevens-Johnson syndrome. Orphanet J Rare Dis. 2010;5:39. doi:10.1186/1750-1172-5-39 National Health Service. Steven Johnsons Syndrome. Ganapati S. Eponymous dermatological signs in bullous dermatoses. Indian J Dermatol. 2014;59(1):21-3. doi:10.4103/0019-5154.123483 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 Additional Reading Mandell: Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases, 7th ed. 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. 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