U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Stevens-Johnson syndrome

MedGen UID:
20955
•Concept ID:
C0038325
•
Disease or Syndrome
Synonyms: Stevens Johnson Syndrome; Stevens-Johnson Syndrome
SNOMED CT: Stevens-Johnson syndrome (73442001); Stevens Johnson syndrome (73442001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018229
Orphanet: ORPHA36426

Definition

Stevens-Johnson syndrome is a limited form of toxic epidermal necrolysis with characteristics of destruction and detachment of the skin epithelium and mucous membranes involving less than 10% of the body surface area. The disease can be triggered by a drug allergy and, more rarely, by infections or bone marrow transplantation. In 25 to 30% of cases, the cause is unclear. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVStevens-Johnson syndrome
Follow this link to review classifications for Stevens-Johnson syndrome in Orphanet.

Professional guidelines

PubMed

Systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome.
Jacobsen A, Olabi B, Langley A, Beecker J, Mutter E, Shelley A, Worley B, Ramsay T, Saavedra A, Parker R, Stewart F, Pardo Pardo J
Cochrane Database Syst Rev 2022 Mar 11;3(3):CD013130. doi: 10.1002/14651858.CD013130.pub2. PMID: 35274741Free PMC Article
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management.
Frantz R, Huang S, Are A, Motaparthi K
Medicina (Kaunas) 2021 Aug 28;57(9) doi: 10.3390/medicina57090895. PMID: 34577817Free PMC Article
Erythema Multiforme: Recognition and Management.
Trayes KP, Love G, Studdiford JS
Am Fam Physician 2019 Jul 15;100(2):82-88. PMID: 31305041

Recent clinical studies

Etiology

Drug Hypersensitivity Reactions.
Wilkerson RG
Immunol Allergy Clin North Am 2023 Aug;43(3):473-489. doi: 10.1016/j.iac.2022.10.005. PMID: 37394254
Sulfonamide Hypersensitivity.
Chow TG, Khan DA
Clin Rev Allergy Immunol 2022 Jun;62(3):400-412. Epub 2021 Jul 1 doi: 10.1007/s12016-021-08872-3. PMID: 34212341
Lamotrigine and Stevens-Johnson Syndrome Prevention.
Edinoff AN, Nguyen LH, Fitz-Gerald MJ, Crane E, Lewis K, Pierre SS, Kaye AD, Kaye AM, Kaye JS, Kaye RJ, Gennuso SA, Varrassi G, Viswanath O, Urits I
Psychopharmacol Bull 2021 Mar 16;51(2):96-114. PMID: 34092825Free PMC Article
Erythema multiforme, Stevens-Johnson syndrome/toxic epidermal necrolysis - diagnosis and treatment.
Grünwald P, Mockenhaupt M, Panzer R, Emmert S
J Dtsch Dermatol Ges 2020 Jun;18(6):547-553. Epub 2020 May 29 doi: 10.1111/ddg.14118. PMID: 32469468
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article

Diagnosis

Drug Hypersensitivity Reactions.
Wilkerson RG
Immunol Allergy Clin North Am 2023 Aug;43(3):473-489. doi: 10.1016/j.iac.2022.10.005. PMID: 37394254
Stevens-Johnson syndrome in children.
Ramien ML
Curr Opin Pediatr 2022 Aug 1;34(4):341-348. doi: 10.1097/MOP.0000000000001146. PMID: 35836393
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management.
Frantz R, Huang S, Are A, Motaparthi K
Medicina (Kaunas) 2021 Aug 28;57(9) doi: 10.3390/medicina57090895. PMID: 34577817Free PMC Article
Pediatric SJS-TEN: Where are we now?
Ramien M, Goldman JL
F1000Res 2020;9 Epub 2020 Aug 13 doi: 10.12688/f1000research.20419.1. PMID: 32850118Free PMC Article
Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis.
Hasegawa A, Abe R
F1000Res 2020;9 Epub 2020 Jun 16 doi: 10.12688/f1000research.24748.1. PMID: 32595945Free PMC Article

Therapy

Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: A Case Report and Literature Review.
Meledathu S, Gordon M, Thornton M, Ashinoff R
J Drugs Dermatol 2023 Nov 1;22(11):e24-e28. doi: 10.36849/JDD.6999. PMID: 37943271
New insights into the diagnosis and management of Stevens-Johnson syndrome and toxic epidermal necrolysis.
Saito Y, Abe R
Curr Opin Allergy Clin Immunol 2023 Aug 1;23(4):271-278. Epub 2023 Jun 6 doi: 10.1097/ACI.0000000000000914. PMID: 37284785
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management.
Frantz R, Huang S, Are A, Motaparthi K
Medicina (Kaunas) 2021 Aug 28;57(9) doi: 10.3390/medicina57090895. PMID: 34577817Free PMC Article
Systemic Immunomodulating Therapies for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis.
Zimmermann S, Sekula P, Venhoff M, Motschall E, Knaus J, Schumacher M, Mockenhaupt M
JAMA Dermatol 2017 Jun 1;153(6):514-522. doi: 10.1001/jamadermatol.2016.5668. PMID: 28329382Free PMC Article
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article

Prognosis

Reactive infectious mucocutaneous eruption: Mycoplasma pneumoniae-induced rash and mucositis and other parainfectious eruptions.
Ramien ML
Clin Exp Dermatol 2021 Apr;46(3):420-429. Epub 2020 Sep 12 doi: 10.1111/ced.14404. PMID: 32918499
Bullous Drug Reactions.
Mockenhaupt M
Acta Derm Venereol 2020 Feb 12;100(5):adv00057. doi: 10.2340/00015555-3408. PMID: 32039459Free PMC Article
Severe cutaneous adverse reactions to drugs.
Duong TA, Valeyrie-Allanore L, Wolkenstein P, Chosidow O
Lancet 2017 Oct 28;390(10106):1996-2011. Epub 2017 May 2 doi: 10.1016/S0140-6736(16)30378-6. PMID: 28476287
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: An Update.
Dodiuk-Gad RP, Chung WH, Valeyrie-Allanore L, Shear NH
Am J Clin Dermatol 2015 Dec;16(6):475-93. doi: 10.1007/s40257-015-0158-0. PMID: 26481651
EMPACT syndrome.
Bilgili SG, Calka O, Karadag AS, Burakgazi AZ
Cutan Ocul Toxicol 2011 Dec;30(4):328-30. Epub 2011 Jun 16 doi: 10.3109/15569527.2011.588298. PMID: 21675930

Clinical prediction guides

Recent advances in managing and understanding Stevens-Johnson syndrome and toxic epidermal necrolysis.
Hasegawa A, Abe R
F1000Res 2020;9 Epub 2020 Jun 16 doi: 10.12688/f1000research.24748.1. PMID: 32595945Free PMC Article
Erythema multiforme, Stevens-Johnson syndrome/toxic epidermal necrolysis - diagnosis and treatment.
Grünwald P, Mockenhaupt M, Panzer R, Emmert S
J Dtsch Dermatol Ges 2020 Jun;18(6):547-553. Epub 2020 May 29 doi: 10.1111/ddg.14118. PMID: 32469468
Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.
Lerch M, Mainetti C, Terziroli Beretta-Piccoli B, Harr T
Clin Rev Allergy Immunol 2018 Feb;54(1):147-176. doi: 10.1007/s12016-017-8654-z. PMID: 29188475
Toxic epidermal necrolysis and Stevens-Johnson syndrome.
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article
ALDEN, an algorithm for assessment of drug causality in Stevens-Johnson Syndrome and toxic epidermal necrolysis: comparison with case-control analysis.
Sassolas B, Haddad C, Mockenhaupt M, Dunant A, Liss Y, Bork K, Haustein UF, Vieluf D, Roujeau JC, Le Louet H
Clin Pharmacol Ther 2010 Jul;88(1):60-8. Epub 2010 Apr 7 doi: 10.1038/clpt.2009.252. PMID: 20375998

Recent systematic reviews

Worldwide Prevalence of Antibiotic-Associated Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis.
Lee EY, Knox C, Phillips EJ
JAMA Dermatol 2023 Apr 1;159(4):384-392. doi: 10.1001/jamadermatol.2022.6378. PMID: 36790777Free PMC Article
Systemic interventions for treatment of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome.
Jacobsen A, Olabi B, Langley A, Beecker J, Mutter E, Shelley A, Worley B, Ramsay T, Saavedra A, Parker R, Stewart F, Pardo Pardo J
Cochrane Database Syst Rev 2022 Mar 11;3(3):CD013130. doi: 10.1002/14651858.CD013130.pub2. PMID: 35274741Free PMC Article
Stevens-Johnson syndrome and toxic epidermal necrolysis: a systematic review and meta-analysis.
Houschyar KS, Tapking C, Borrelli MR, Puladi B, Ooms M, Wallner C, Duscher D, Pförringer D, Rein S, Reumuth G, Schulz T, Nietzschmann I, Maan ZN, Grieb G, Philipp-Dormston WG, Branski LK, Siemers F, Lehnhardt M, Schmitt L, Yazdi AS
J Wound Care 2021 Dec 2;30(12):1012-1019. doi: 10.12968/jowc.2021.30.12.1012. PMID: 34881995
Systemic Immunomodulating Therapies for Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Meta-analysis.
Zimmermann S, Sekula P, Venhoff M, Motschall E, Knaus J, Schumacher M, Mockenhaupt M
JAMA Dermatol 2017 Jun 1;153(6):514-522. doi: 10.1001/jamadermatol.2016.5668. PMID: 28329382Free PMC Article
Mycoplasma pneumoniae-induced rash and mucositis as a syndrome distinct from Stevens-Johnson syndrome and erythema multiforme: a systematic review.
Canavan TN, Mathes EF, Frieden I, Shinkai K
J Am Acad Dermatol 2015 Feb;72(2):239-45. doi: 10.1016/j.jaad.2014.06.026. PMID: 25592340

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...