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Lyell syndrome

MedGen UID:
977858
•Concept ID:
CN294179
•
Disease or Syndrome
Synonym: Toxic epidermal necrolysis
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:0018890
Orphanet: ORPHA537

Definition

An extended form of Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome characterized by destruction and detachment of the skin epithelium and mucous membranes involving more than 30% of the body surface area. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLyell syndrome

Professional guidelines

PubMed

Management of ocular involvement in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: french national audit of practices, literature review, and consensus agreement.
Thorel D, Ingen-Housz-Oro S, Royer G, Delcampe A, Bellon N, Bodemer C, Welfringer-Morin A, Bremond-Gignac D, Robert MP, Tauber M, Malecaze F, Dereure O, Daien V, Colin A, Bernier C, Couret C, Vabres B, Tetart F, Milpied B, Cornut T, Ben Said B, Burillon C, Cordel N, Beral L, de Prost N, Wolkenstein P, Muraine M, Gueudry J
Orphanet J Rare Dis 2020 Sep 22;15(1):259. doi: 10.1186/s13023-020-01538-x. PMID: 32962748Free PMC Article
Toxic epidermal necrolysis (TEN): Acute complications and long-term sequelae management in a multidisciplinary follow-up.
Cabañas Weisz LM, Miguel Escuredo I, Ayestarán Soto JB, García Gutiérrez JJ
J Plast Reconstr Aesthet Surg 2020 Feb;73(2):319-327. Epub 2019 Aug 8 doi: 10.1016/j.bjps.2019.07.015. PMID: 31481319
Inpatient Treatment for Severe Nonsurgical Dermatological Disorders: Prevalence, Care Infrastructure and Reimbursement in Switzerland.
Mehra T, Hoetzenecker W, Moos R, Volbracht J, Guenova E, French LE, Hafner J
Dermatology 2015;231(3):260-8. Epub 2015 Aug 19 doi: 10.1159/000437223. PMID: 26303020

Recent clinical studies

Etiology

Nutritional and metabolic characteristics of critically ill patients admitted for severe toxidermia.
Karakus M, Pantet O, Charrière M, Favre D, Gaide O, Berger MM
Clin Nutr 2023 Jun;42(6):859-868. Epub 2023 Apr 8 doi: 10.1016/j.clnu.2023.04.006. PMID: 37086614
Skin Testing and Drug Provocation Tests in Epidermal Necrolysis: A French Experience.
Roux C, Ben Said B, Milpied B, Bernier C, Staumont-Sallé D, Dezoteux F, Soria A, Barbaud A, Valeyrie-Allanore L, Tétart F, Bellon N, Lebrun-Vignes B, Gener G, Paul M, Ingen-Housz-Oro S, Assier H
J Allergy Clin Immunol Pract 2022 Dec;10(12):3252-3261.e2. Epub 2022 Jul 20 doi: 10.1016/j.jaip.2022.07.008. PMID: 35870724
Severe Cutaneous Adverse Drug Reactions in Children: Epidemiological, Clinical and Etiological Aspects in Dermatology-Venereology Unit at National and Teaching Hospital of Cotonou.
Dégboé B, Koudoukpo C, Almeida CD, Kouassi A, Nguessie C, Akpadjan F, Adégbidi H, Atadokpèdé F
West Afr J Med 2022 May 27;39(5):538-542. PMID: 35633648
Management of ocular involvement in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: french national audit of practices, literature review, and consensus agreement.
Thorel D, Ingen-Housz-Oro S, Royer G, Delcampe A, Bellon N, Bodemer C, Welfringer-Morin A, Bremond-Gignac D, Robert MP, Tauber M, Malecaze F, Dereure O, Daien V, Colin A, Bernier C, Couret C, Vabres B, Tetart F, Milpied B, Cornut T, Ben Said B, Burillon C, Cordel N, Beral L, de Prost N, Wolkenstein P, Muraine M, Gueudry J
Orphanet J Rare Dis 2020 Sep 22;15(1):259. doi: 10.1186/s13023-020-01538-x. PMID: 32962748Free PMC Article
The evil of the unknown--risk-benefit evaluation of new synthetic drugs in the 19th century.
Schneider A, Helmstädter A
Pharmazie 2015 Jan;70(1):60-3. PMID: 25975100

Diagnosis

Stevens-Johnson syndrome and toxic epidermal necrolysis from pathologist's point of view.
Sticová E, Kyclová J, Důra M, Štork J, Lipový B
Cesk Patol 2023 Fall;59(3):124-128. PMID: 37805268
Skin Testing and Drug Provocation Tests in Epidermal Necrolysis: A French Experience.
Roux C, Ben Said B, Milpied B, Bernier C, Staumont-Sallé D, Dezoteux F, Soria A, Barbaud A, Valeyrie-Allanore L, Tétart F, Bellon N, Lebrun-Vignes B, Gener G, Paul M, Ingen-Housz-Oro S, Assier H
J Allergy Clin Immunol Pract 2022 Dec;10(12):3252-3261.e2. Epub 2022 Jul 20 doi: 10.1016/j.jaip.2022.07.008. PMID: 35870724
Severe Cutaneous Adverse Drug Reactions in Children: Epidemiological, Clinical and Etiological Aspects in Dermatology-Venereology Unit at National and Teaching Hospital of Cotonou.
Dégboé B, Koudoukpo C, Almeida CD, Kouassi A, Nguessie C, Akpadjan F, Adégbidi H, Atadokpèdé F
West Afr J Med 2022 May 27;39(5):538-542. PMID: 35633648
Vajra Bhasma, Ayurvedic medicine: a rare and unusual cause of Lyell's syndrome and its successful management.
Channabasappa SM, Gupta B, Dharmappa S
BMJ Case Rep 2020 Oct 30;13(10) doi: 10.1136/bcr-2020-237891. PMID: 33127708Free PMC Article
Epidermal necrolysis French national diagnosis and care protocol (PNDS; protocole national de diagnostic et de soins).
Ingen-Housz-Oro S, Duong TA, Bensaid B, Bellon N, de Prost N, Lu D, Lebrun-Vignes B, Gueudry J, Bequignon E, Zaghbib K, Royer G, Colin A, Do-Pham G, Bodemer C, Ortonne N, Barbaud A, Fardet L, Chosidow O, Wolkenstein P; French National Reference Center for Toxic Bullous Dermatoses
Orphanet J Rare Dis 2018 Apr 10;13(1):56. doi: 10.1186/s13023-018-0793-7. PMID: 29636107Free PMC Article

Therapy

Skin Testing and Drug Provocation Tests in Epidermal Necrolysis: A French Experience.
Roux C, Ben Said B, Milpied B, Bernier C, Staumont-Sallé D, Dezoteux F, Soria A, Barbaud A, Valeyrie-Allanore L, Tétart F, Bellon N, Lebrun-Vignes B, Gener G, Paul M, Ingen-Housz-Oro S, Assier H
J Allergy Clin Immunol Pract 2022 Dec;10(12):3252-3261.e2. Epub 2022 Jul 20 doi: 10.1016/j.jaip.2022.07.008. PMID: 35870724
Therapeutic approach of Lyell syndrome with infliximab and dexamethasone pulse: report of a clinical case.
Pacheco JDJ, Ribeiro MRV, Queirós CSDS, Xambre MFCM
An Bras Dermatol 2022 May-Jun;97(3):402-404. Epub 2022 Apr 2 doi: 10.1016/j.abd.2020.10.020. PMID: 35379509Free PMC Article
Vajra Bhasma, Ayurvedic medicine: a rare and unusual cause of Lyell's syndrome and its successful management.
Channabasappa SM, Gupta B, Dharmappa S
BMJ Case Rep 2020 Oct 30;13(10) doi: 10.1136/bcr-2020-237891. PMID: 33127708Free PMC Article
Management of ocular involvement in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: french national audit of practices, literature review, and consensus agreement.
Thorel D, Ingen-Housz-Oro S, Royer G, Delcampe A, Bellon N, Bodemer C, Welfringer-Morin A, Bremond-Gignac D, Robert MP, Tauber M, Malecaze F, Dereure O, Daien V, Colin A, Bernier C, Couret C, Vabres B, Tetart F, Milpied B, Cornut T, Ben Said B, Burillon C, Cordel N, Beral L, de Prost N, Wolkenstein P, Muraine M, Gueudry J
Orphanet J Rare Dis 2020 Sep 22;15(1):259. doi: 10.1186/s13023-020-01538-x. PMID: 32962748Free PMC Article
Effectiveness of Suprathel® application in partial thickness burns, frostbites and Lyell syndrome treatment.
Mądry R, Strużyna J, Stachura-Kułach A, Drozdz Ł, Bugaj M
Pol Przegl Chir 2011 Oct;83(10):541-8. doi: 10.2478/v10035-011-0086-5. PMID: 22189281

Prognosis

End-Stage Respiratory Failure Secondary to Bronchiolitis Obliterans Syndrome Induced by Toxic Epidermal Necrosis, Also Known as Lyell Syndrome: A Case Report.
Matar M, Kessler R, Olland A, Falcoz P, Desprez P, Roche A, Collange O, Chenard MP, Renaud-Picard B, Porzio M
Transplant Proc 2021 May;53(4):1371-1374. Epub 2021 Apr 23 doi: 10.1016/j.transproceed.2021.03.020. PMID: 33896544
Management of ocular involvement in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: french national audit of practices, literature review, and consensus agreement.
Thorel D, Ingen-Housz-Oro S, Royer G, Delcampe A, Bellon N, Bodemer C, Welfringer-Morin A, Bremond-Gignac D, Robert MP, Tauber M, Malecaze F, Dereure O, Daien V, Colin A, Bernier C, Couret C, Vabres B, Tetart F, Milpied B, Cornut T, Ben Said B, Burillon C, Cordel N, Beral L, de Prost N, Wolkenstein P, Muraine M, Gueudry J
Orphanet J Rare Dis 2020 Sep 22;15(1):259. doi: 10.1186/s13023-020-01538-x. PMID: 32962748Free PMC Article
Incidence of bloodstream infections and predictive value of qualitative and quantitative skin cultures of patients with overlap syndrome or toxic epidermal necrolysis: A retrospective observational cohort study of 98 cases.
Lecadet A, Woerther PL, Hua C, Colin A, Gomart C, Decousser JW, Mekontso Dessap A, Wolkenstein P, Chosidow O, de Prost N, Ingen-Housz-Oro S
J Am Acad Dermatol 2019 Aug;81(2):342-347. Epub 2019 Mar 16 doi: 10.1016/j.jaad.2019.03.030. PMID: 30890340
Toxic epidermal necrolysis (Lyell syndrome): more than "acute skin failure".
Roujeau JC
Intensive Care Med 1992;18(1):4-5. doi: 10.1007/BF01706417. PMID: 1578046
Toxic epidermal necrolysis (Lyell syndrome).
Roujeau JC, Chosidow O, Saiag P, Guillaume JC
J Am Acad Dermatol 1990 Dec;23(6 Pt 1):1039-58. doi: 10.1016/0190-9622(90)70333-d. PMID: 2273103

Clinical prediction guides

Nutritional and metabolic characteristics of critically ill patients admitted for severe toxidermia.
Karakus M, Pantet O, Charrière M, Favre D, Gaide O, Berger MM
Clin Nutr 2023 Jun;42(6):859-868. Epub 2023 Apr 8 doi: 10.1016/j.clnu.2023.04.006. PMID: 37086614
Skin Testing and Drug Provocation Tests in Epidermal Necrolysis: A French Experience.
Roux C, Ben Said B, Milpied B, Bernier C, Staumont-Sallé D, Dezoteux F, Soria A, Barbaud A, Valeyrie-Allanore L, Tétart F, Bellon N, Lebrun-Vignes B, Gener G, Paul M, Ingen-Housz-Oro S, Assier H
J Allergy Clin Immunol Pract 2022 Dec;10(12):3252-3261.e2. Epub 2022 Jul 20 doi: 10.1016/j.jaip.2022.07.008. PMID: 35870724
End-Stage Respiratory Failure Secondary to Bronchiolitis Obliterans Syndrome Induced by Toxic Epidermal Necrosis, Also Known as Lyell Syndrome: A Case Report.
Matar M, Kessler R, Olland A, Falcoz P, Desprez P, Roche A, Collange O, Chenard MP, Renaud-Picard B, Porzio M
Transplant Proc 2021 May;53(4):1371-1374. Epub 2021 Apr 23 doi: 10.1016/j.transproceed.2021.03.020. PMID: 33896544
Incidence of bloodstream infections and predictive value of qualitative and quantitative skin cultures of patients with overlap syndrome or toxic epidermal necrolysis: A retrospective observational cohort study of 98 cases.
Lecadet A, Woerther PL, Hua C, Colin A, Gomart C, Decousser JW, Mekontso Dessap A, Wolkenstein P, Chosidow O, de Prost N, Ingen-Housz-Oro S
J Am Acad Dermatol 2019 Aug;81(2):342-347. Epub 2019 Mar 16 doi: 10.1016/j.jaad.2019.03.030. PMID: 30890340
Toxic epidermal necrolysis (Lyell syndrome).
Roujeau JC, Chosidow O, Saiag P, Guillaume JC
J Am Acad Dermatol 1990 Dec;23(6 Pt 1):1039-58. doi: 10.1016/0190-9622(90)70333-d. PMID: 2273103

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