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Erythema multiforme major

MedGen UID:
469028
Concept ID:
C3241919
Disease or Syndrome
Synonyms: Erythema exsudativum multiforme majus; erythema exsudativum multiforme majus; Erythema Multiforme Major; erythema multiforme major; erythema multiforme majus; Erythema multiforme majus
SNOMED CT: Erythema multiforme major (16898231000119107)
 
Monarch Initiative: MONDO:0044719
Orphanet: ORPHA502499

Definition

A rare skin disease characterized most typically by targetoid papules with concentric color variation symmetrically distributed on the extensor surfaces of the extremities, accompanied by mucosal involvement (in particular the oral mucosa) in the form of initial erythema with edema, progressing to superficial erosions with pseudomembrane formation. Grouping of lesions around the elbows and knees and edema of the nail folds may also be observed. The condition is commonly proceeded by prodromal symptoms of malaise, fever, and myalgias, and is usually self-limiting, although recurrent disease is seen in a subset of patients. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVErythema multiforme major

Professional guidelines

PubMed

Schwartz RA, McDonough PH, Lee BW
J Am Acad Dermatol 2013 Aug;69(2):187.e1-16; quiz 203-4. doi: 10.1016/j.jaad.2013.05.002. PMID: 23866879
Bachot N, Roujeau JC
Am J Clin Dermatol 2003;4(8):561-72. doi: 10.2165/00128071-200304080-00006. PMID: 12862499
Raviglione MC, Pablos-Mendez A, Battan R
Drug Saf 1990 Jan-Feb;5(1):39-64. doi: 10.2165/00002018-199005010-00005. PMID: 2138020

Recent clinical studies

Etiology

Giraud-Kerleroux L, Bellon N, Welfringer-Morin A, Leclerc-Mercier S, Costedoat I, Coquin J, Brun A, Roguedas-Contios AM, Bernier C, Milpied B, Tétart F, Du Thanh A, Cordel N, Bensaid B, Fargeas C, Tauber M, Renolleau S, Boralevi F, Ingen-Housz-Oro S, Bodemer C
J Eur Acad Dermatol Venereol 2021 Oct;35(10):2051-2058. Epub 2021 Jul 21 doi: 10.1111/jdv.17469. PMID: 34157175
Gupta SS, Sabharwal N, Patti R, Kupfer Y
Am J Med Sci 2019 Apr;357(4):348-351. Epub 2018 Dec 4 doi: 10.1016/j.amjms.2018.11.018. PMID: 30638600
Heng YK, Lee HY, Roujeau JC
Br J Dermatol 2015 Nov;173(5):1250-4. doi: 10.1111/bjd.13989. PMID: 26769645
Schwartz RA, McDonough PH, Lee BW
J Am Acad Dermatol 2013 Aug;69(2):187.e1-16; quiz 203-4. doi: 10.1016/j.jaad.2013.05.002. PMID: 23866879
Huff JC
Dermatol Clin 1985 Jan;3(1):141-52. PMID: 4092378

Diagnosis

Hattori S, Miyagawa F, Fukuda K, Ogawa K, Asada H
Acta Derm Venereol 2021 Dec 13;101(12):adv00616. doi: 10.2340/actadv.v101.165. PMID: 34877607Free PMC Article
Errichetti E, Stinco G
Clin Dermatol 2020 Jan-Feb;38(1):113-121. Epub 2019 Nov 11 doi: 10.1016/j.clindermatol.2019.10.013. PMID: 32197741
Heng YK, Lee HY, Roujeau JC
Br J Dermatol 2015 Nov;173(5):1250-4. doi: 10.1111/bjd.13989. PMID: 26769645
Stampien TM, Schwartz RA
Am Fam Physician 1992 Oct;46(4):1171-6. PMID: 1414882
Huff JC
Dermatol Clin 1985 Jan;3(1):141-52. PMID: 4092378

Therapy

Yamada M, Furuta M, Fujimoto N
Eur J Dermatol 2023 Aug 1;33(4):437-439. doi: 10.1684/ejd.2023.4520. PMID: 37823501
Gupta SS, Sabharwal N, Patti R, Kupfer Y
Am J Med Sci 2019 Apr;357(4):348-351. Epub 2018 Dec 4 doi: 10.1016/j.amjms.2018.11.018. PMID: 30638600
Sebastian A, Patterson C, Zaenglein AL, Ioffreda MD, Helm KF
J Cutan Pathol 2009 Dec;36(12):1323-5. doi: 10.1111/j.1600-0560.2009.01287.x. PMID: 19302571
Stampien TM, Schwartz RA
Am Fam Physician 1992 Oct;46(4):1171-6. PMID: 1414882
Huff JC
Dermatol Clin 1985 Jan;3(1):141-52. PMID: 4092378

Prognosis

Heng YK, Lee HY, Roujeau JC
Br J Dermatol 2015 Nov;173(5):1250-4. doi: 10.1111/bjd.13989. PMID: 26769645
Brănişteanu DE, Voicu CM, Creţu A, Dimitriu A, Luca MC, Sălăvăstru CM
Rev Med Chir Soc Med Nat Iasi 2015 Jan-Mar;119(1):38-44. PMID: 25970940
Schwartz RA, McDonough PH, Lee BW
J Am Acad Dermatol 2013 Aug;69(2):187.e1-16; quiz 203-4. doi: 10.1016/j.jaad.2013.05.002. PMID: 23866879
Johnston GA, Ghura HS, Carter E, Graham-Brown RA
Clin Exp Dermatol 2002 Nov;27(8):661-4. doi: 10.1046/j.1365-2230.2002.01098.x. PMID: 12472541
Kakourou T, Klontza D, Soteropoulou F, Kattamis C
Eur J Pediatr 1997 Feb;156(2):90-3. doi: 10.1007/s004310050561. PMID: 9039508

Clinical prediction guides

Liu RF, Chen CB, Hui RCY, Kuan YZ, Chung WH
J Dermatol Sci 2018 Oct;92(1):38-44. Epub 2018 Aug 8 doi: 10.1016/j.jdermsci.2018.08.002. PMID: 30143369
Ellis E, Sidhu S
Australas J Dermatol 2014 Nov;55(4):266-9. Epub 2014 Apr 1 doi: 10.1111/ajd.12162. PMID: 24689866
Schwartz RA, McDonough PH, Lee BW
J Am Acad Dermatol 2013 Aug;69(2):187.e1-16; quiz 203-4. doi: 10.1016/j.jaad.2013.05.002. PMID: 23866879
Micali G, Linthicum K, Han N, West DP
Pharmacotherapy 1999 Feb;19(2):223-7. doi: 10.1592/phco.19.3.223.30917. PMID: 10030773
Hunziker T, Künzi UP, Braunschweig S, Zehnder D, Hoigné R
Allergy 1997 Apr;52(4):388-93. doi: 10.1111/j.1398-9995.1997.tb01017.x. PMID: 9188919

Recent systematic reviews

Schneider G, Kachroo S, Jones N, Crean S, Rotella P, Avetisyan R, Reynolds MW
Pharmacoepidemiol Drug Saf 2012 Jan;21 Suppl 1:236-9. doi: 10.1002/pds.2331. PMID: 22262611

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