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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.
 
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

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
Frantz R, Huang S, Are A, Motaparthi K
Medicina (Kaunas) 2021 Aug 28;57(9) doi: 10.3390/medicina57090895. PMID: 34577817Free PMC Article
Trayes KP, Love G, Studdiford JS
Am Fam Physician 2019 Jul 15;100(2):82-88. PMID: 31305041

Recent clinical studies

Etiology

Wilkerson RG
Immunol Allergy Clin North Am 2023 Aug;43(3):473-489. doi: 10.1016/j.iac.2022.10.005. PMID: 37394254
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
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
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
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

Ramien ML
Curr Opin Pediatr 2022 Aug 1;34(4):341-348. doi: 10.1097/MOP.0000000000001146. PMID: 35836393
Frantz R, Huang S, Are A, Motaparthi K
Medicina (Kaunas) 2021 Aug 28;57(9) doi: 10.3390/medicina57090895. PMID: 34577817Free PMC Article
Ramien M, Goldman JL
F1000Res 2020;9 Epub 2020 Aug 13 doi: 10.12688/f1000research.20419.1. PMID: 32850118Free PMC Article
Hasegawa A, Abe R
F1000Res 2020;9 Epub 2020 Jun 16 doi: 10.12688/f1000research.24748.1. PMID: 32595945Free PMC Article
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

Therapy

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
Singh N, Phillips M
Skin Therapy Lett 2022 Sep;27(5):7-13. PMID: 36469487
Frantz R, Huang S, Are A, Motaparthi K
Medicina (Kaunas) 2021 Aug 28;57(9) doi: 10.3390/medicina57090895. PMID: 34577817Free PMC Article
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
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

Mockenhaupt M
Acta Derm Venereol 2020 Feb 12;100(5):adv00057. doi: 10.2340/00015555-3408. PMID: 32039459Free PMC Article
Park JW, Curtis JR, Moon J, Song YW, Kim S, Lee EB
Ann Rheum Dis 2018 May;77(5):644-649. Epub 2017 Nov 1 doi: 10.1136/annrheumdis-2017-211796. PMID: 29092853Free PMC Article
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
Wong A, Malvestiti AA, Hafner Mde F
Rev Assoc Med Bras (1992) 2016 Sep-Oct;62(5):468-73. doi: 10.1590/1806-9282.62.05.468. PMID: 27656858
Bastuji-Garin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Wolkenstein P
J Invest Dermatol 2000 Aug;115(2):149-53. doi: 10.1046/j.1523-1747.2000.00061.x. PMID: 10951229

Clinical prediction guides

Hasegawa A, Abe R
F1000Res 2020;9 Epub 2020 Jun 16 doi: 10.12688/f1000research.24748.1. PMID: 32595945Free PMC Article
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
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
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
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article

Recent systematic reviews

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
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
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
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
Nanau RM, Neuman MG
Clin Biochem 2013 Oct;46(15):1323-38. Epub 2013 Jun 20 doi: 10.1016/j.clinbiochem.2013.06.012. PMID: 23792104

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