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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

Respiratory complications in pediatric epidermal necrolysis: A retrospective study of 22 cases.
Schmartz S, Welfringer-Morin A, Le Bourgeois M, Delacourt C, Berteloot L, Rabia SH, Bellon N, Moulin F, Pouletty M, Garcelon N, Bodemer C, Drummond D
J Am Acad Dermatol 2023 Oct;89(4):857-859. Epub 2023 Jun 23 doi: 10.1016/j.jaad.2023.05.092. PMID: 37355226
Effectiveness of seasonal malaria chemoprevention in three regions of Togo: a population-based longitudinal study from 2013 to 2020.
Bakai TA, Thomas A, Iwaz J, Atcha-Oubou T, Tchadjobo T, Khanafer N, Rabilloud M, Voirin N
Malar J 2022 Dec 31;21(1):400. doi: 10.1186/s12936-022-04434-w. PMID: 36587191Free 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
Etofenamate associated with Lyell syndrome: a case report.
Golcuk Y, Oray D, Atilla OD, Tefennioglu N
Clin Toxicol (Phila) 2010 Jun;48(5):471-2. doi: 10.3109/15563651003796374. PMID: 20586575
Gemcitabine: cutaneous, hepatic and vascular disorders.
Prescrire Int 2009 Jun;18(101):122. PMID: 19637433

Diagnosis

S3 guideline: Diagnosis and treatment of epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis) - Part 1: Diagnosis, initial management, and immunomodulating systemic therapy.
Heuer R, Paulmann M, Annecke T, Behr B, Boch K, Boos AM, Brockow K, French LE, Gille J, Gundlach V, Hartmann B, Höger P, Hofmann SC, Klein T, Lehnhardt M, Liß Y, Maier P, Mandel P, Marathovouniotis N, Marlok F, Mittelviefhaus H, Pleyer U, Pradeau M, Rall K, Rieg S, Rittner H, Sander F, Schnitzler S, Schut C, Stolle A, Vorobyev A, Wedi B, Weiss J, Zepp M, Ziemer M, Mockenhaupt M, Nast A
J Dtsch Dermatol Ges 2024 Oct;22(10):1448-1466. Epub 2024 Sep 23 doi: 10.1111/ddg.15515. PMID: 39314017
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
Respiratory complications in pediatric epidermal necrolysis: A retrospective study of 22 cases.
Schmartz S, Welfringer-Morin A, Le Bourgeois M, Delacourt C, Berteloot L, Rabia SH, Bellon N, Moulin F, Pouletty M, Garcelon N, Bodemer C, Drummond D
J Am Acad Dermatol 2023 Oct;89(4):857-859. Epub 2023 Jun 23 doi: 10.1016/j.jaad.2023.05.092. PMID: 37355226
Toxic epidermal necrolysis (Lyell syndrome) in 77 elderly patients.
Bastuji-Garin S, Zahedi M, Guillaume JC, Roujeau JC
Age Ageing 1993 Nov;22(6):450-6. doi: 10.1093/ageing/22.6.450. PMID: 8310891
Acute epidermal necrolysis or Lyell syndrome.
Sluysmans T, De Bont B, Cornu G
Eur J Pediatr 1987 Mar;146(2):199-200. doi: 10.1007/BF02343236. PMID: 3569362

Therapy

Effectiveness of seasonal malaria chemoprevention in three regions of Togo: a population-based longitudinal study from 2013 to 2020.
Bakai TA, Thomas A, Iwaz J, Atcha-Oubou T, Tchadjobo T, Khanafer N, Rabilloud M, Voirin N
Malar J 2022 Dec 31;21(1):400. doi: 10.1186/s12936-022-04434-w. PMID: 36587191Free PMC Article
Toxic epidermal necrolysis complicating antibiotic treatment of puerperal endometritis: a case report.
Simetka O, Michalec I, Crkvenjas ZN, Klosova H, Janackova L, Klat J
Ginekol Pol 2015 Apr;86(4):315-7. doi: 10.17772/gp/2080. PMID: 26117993
Lyell syndrome due to ticlopidine.
Strippoli D, Russo G, Simonetti V, Motolese A
G Ital Dermatol Venereol 2011 Dec;146(6):497-500. PMID: 22095182
Gemcitabine: cutaneous, hepatic and vascular disorders.
Prescrire Int 2009 Jun;18(101):122. PMID: 19637433
Drug-induced Lyell and Stevens-Johnson syndromes.
Prescrire Int 2009 Feb;18(99):20-2. PMID: 19391288

Prognosis

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
Toxic epidermal necrolysis complicating antibiotic treatment of puerperal endometritis: a case report.
Simetka O, Michalec I, Crkvenjas ZN, Klosova H, Janackova L, Klat J
Ginekol Pol 2015 Apr;86(4):315-7. doi: 10.17772/gp/2080. PMID: 26117993
Toxic epidermal necrolysis (Lyell syndrome) in 77 elderly patients.
Bastuji-Garin S, Zahedi M, Guillaume JC, Roujeau JC
Age Ageing 1993 Nov;22(6):450-6. doi: 10.1093/ageing/22.6.450. PMID: 8310891
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
Immunoelectrophoretic changes in Lyell syndrome.
Husz S, Berkó G, Schneider I, Simon N
Int J Dermatol 1974 Jul-Aug;13(4):205-9. doi: 10.1111/j.1365-4362.1974.tb01794.x. PMID: 4139961

Clinical prediction guides

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
Toxic epidermal necrolysis complicating antibiotic treatment of puerperal endometritis: a case report.
Simetka O, Michalec I, Crkvenjas ZN, Klosova H, Janackova L, Klat J
Ginekol Pol 2015 Apr;86(4):315-7. doi: 10.17772/gp/2080. PMID: 26117993
Oseltamivir: cutaneous and neurological adverse effects in children.
Prescrire Int 2006 Oct;15(85):182-3. PMID: 17128526
Ileal involvement in toxic epidermal necrolysis (Lyell syndrome).
Michel P, Joly P, Ducrotte P, Hemet J, Leblanc I, Lauret P, Lerebours E, Colin R
Dig Dis Sci 1993 Oct;38(10):1938-41. doi: 10.1007/BF01296123. PMID: 8404419
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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