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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.
 
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

Abedi F, Zarei B, Elyasi S
Eur J Clin Pharmacol 2024 Aug;80(8):1151-1169. Epub 2024 Apr 12 doi: 10.1007/s00228-024-03664-y. PMID: 38607390
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

Recent clinical studies

Etiology

Wilkerson RG
Emerg Med Clin North Am 2022 Feb;40(1):39-55. Epub 2021 Oct 29 doi: 10.1016/j.emc.2021.09.001. PMID: 34782090
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
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
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

Diagnosis

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
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

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
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
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
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

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
Singh N, Phillips M
Skin Therapy Lett 2022 Sep;27(5):7-13. PMID: 36469487
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
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

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
Harr T, French LE
Orphanet J Rare Dis 2010 Dec 16;5:39. doi: 10.1186/1750-1172-5-39. PMID: 21162721Free PMC Article
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

Stewart TJ, Farrell J, Frew JW
Australas J Dermatol 2024 Sep;65(6):491-504. Epub 2024 Jun 3 doi: 10.1111/ajd.14329. PMID: 38831709
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

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