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Toxic epidermal necrolysis

MedGen UID:
4501
•Concept ID:
C0014518
•
Disease or Syndrome
Synonyms: Mucocutaneous exfoliative disease; TOXIC EPIDERMAL NECROLYSIS, SUSCEPTIBILITY TO
SNOMED CT: Toxic epidermal necrolysis (768962006); Lyell syndrome (768962006)
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:0019810
Orphanet: ORPHA95455

Definition

An extended form of toxic epidermal necrolysis with characteristics of destruction and detachment of the skin epithelium and mucous membranes involving more than 30% of the body surface area. Lyell syndrome can be triggered by a drug allergy and, exceptionally, by infections or bone marrow transplantation. In 25 to 30% of cases, the cause is unclear. Patients should be admitted to an intensive care or burns unit as soon as the diagnosis is suspected. Prognosis is poor (mortality rate: 20-25%). [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Toxic epidermal necrolysis
Follow this link to review classifications for Toxic epidermal necrolysis 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
Adverse cutaneous toxicities by PD-1/PD-L1 immune checkpoint inhibitors: pathogenesis, treatment, and surveillance.
Bhardwaj M, Chiu MN, Pilkhwal Sah S
Cutan Ocul Toxicol 2022 Mar;41(1):73-90. Epub 2022 Feb 20 doi: 10.1080/15569527.2022.2034842. PMID: 35107396
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

Recent clinical studies

Etiology

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
Prevention of Drug Hypersensitivity Reactions: Prescreening and Premedication.
Thong BY, Vultaggio A, Rerkpattanapipat T, Schrijvers R
J Allergy Clin Immunol Pract 2021 Aug;9(8):2958-2966. doi: 10.1016/j.jaip.2021.04.006. PMID: 34366094
Current Perspectives on Severe Drug Eruption.
Zhang J, Lei Z, Xu C, Zhao J, Kang X
Clin Rev Allergy Immunol 2021 Dec;61(3):282-298. Epub 2021 Jul 17 doi: 10.1007/s12016-021-08859-0. PMID: 34273058Free 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

Diagnosis

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
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
Clinical features, outcomes and treatment in children with drug induced Stevens-Johnson syndrome and toxic epidermal necrolysis.
Liotti L, Caimmi S, Bottau P, Bernardini R, Cardinale F, Saretta F, Mori F, Crisafulli G, Franceschini F, Caffarelli C
Acta Biomed 2019 Jan 29;90(3-S):52-60. doi: 10.23750/abm.v90i3-S.8165. PMID: 30830062Free PMC Article

Therapy

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
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
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
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

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
Clinical Phenotypes of Severe Cutaneous Drug Hypersensitivity Reactions.
Guvenir H, Arikoglu T, Vezir E, Misirlioglu ED
Curr Pharm Des 2019;25(36):3840-3854. doi: 10.2174/1381612825666191107162921. PMID: 31696807
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
SCORTEN: a severity-of-illness score for toxic epidermal necrolysis.
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

Cutaneous adverse events caused by immune checkpoint inhibitors.
Quach HT, Johnson DB, LeBoeuf NR, Zwerner JP, Dewan AK
J Am Acad Dermatol 2021 Oct;85(4):956-966. Epub 2021 Jul 28 doi: 10.1016/j.jaad.2020.09.054. PMID: 34332798
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

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
Adverse drug reactions induced by valproic acid.
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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