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Stevens-Johnson syndrome

MedGen UID:
20955
•Concept ID:
C0038325
•
Disease or Syndrome
Synonyms: Stevens Johnson Syndrome; Stevens-Johnson Syndrome
SNOMED CT: Stevens-Johnson syndrome (73442001); Stevens Johnson syndrome (73442001)
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:0018229
Orphanet: ORPHA36426

Definition

Stevens-Johnson syndrome is a limited form of toxic epidermal necrolysis with characteristics of destruction and detachment of the skin epithelium and mucous membranes involving less than 10% of the body surface area. The disease can be triggered by a drug allergy and, more rarely, by infections or bone marrow transplantation. In 25 to 30% of cases, the cause is unclear. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVStevens-Johnson syndrome
Follow this link to review classifications for Stevens-Johnson syndrome in Orphanet.

Professional guidelines

PubMed

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
Erythema Multiforme: Recognition and Management.
Trayes KP, Love G, Studdiford JS
Am Fam Physician 2019 Jul 15;100(2):82-88. PMID: 31305041

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
Lamotrigine and Stevens-Johnson Syndrome Prevention.
Edinoff AN, Nguyen LH, Fitz-Gerald MJ, Crane E, Lewis K, Pierre SS, Kaye AD, Kaye AM, Kaye JS, Kaye RJ, Gennuso SA, Varrassi G, Viswanath O, Urits I
Psychopharmacol Bull 2021 Mar 16;51(2):96-114. PMID: 34092825Free PMC Article
Diagnosis and management of Stevens-Johnson syndrome/toxic epidermal necrolysis.
Noe MH, Micheletti RG
Clin Dermatol 2020 Nov-Dec;38(6):607-612. Epub 2020 Jun 30 doi: 10.1016/j.clindermatol.2020.06.016. PMID: 33341195
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
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

Diagnosis

Stevens-Johnson syndrome in children.
Ramien ML
Curr Opin Pediatr 2022 Aug 1;34(4):341-348. doi: 10.1097/MOP.0000000000001146. PMID: 35836393
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 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
Stevens-Johnson syndrome.
Bakshi SS
Intern Emerg Med 2019 Mar;14(2):323-324. Epub 2018 Nov 8 doi: 10.1007/s11739-018-1981-0. PMID: 30406877

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
Oral isotretinoin for acne.
Costa CS, Bagatin E, Martimbianco ALC, da Silva EM, Lúcio MM, Magin P, Riera R
Cochrane Database Syst Rev 2018 Nov 24;11(11):CD009435. doi: 10.1002/14651858.CD009435.pub2. PMID: 30484286Free PMC Article
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

Bullous Drug Reactions.
Mockenhaupt M
Acta Derm Venereol 2020 Feb 12;100(5):adv00057. doi: 10.2340/00015555-3408. PMID: 32039459Free PMC Article
Prophylactic effect of trimethoprim-sulfamethoxazole for pneumocystis pneumonia in patients with rheumatic diseases exposed to prolonged high-dose glucocorticoids.
Park JW, Curtis JR, Moon J, Song YW, Kim S, Lee EB
Ann Rheum Dis 2018 May;77(5):644-649. Epub 2017 Nov 1 doi: 10.1136/annrheumdis-2017-211796. PMID: 29092853Free PMC Article
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
Stevens-Johnson syndrome and toxic epidermal necrolysis: a review.
Wong A, Malvestiti AA, Hafner Mde F
Rev Assoc Med Bras (1992) 2016 Sep-Oct;62(5):468-73. doi: 10.1590/1806-9282.62.05.468. PMID: 27656858
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: An Update.
Dodiuk-Gad RP, Chung WH, Valeyrie-Allanore L, Shear NH
Am J Clin Dermatol 2015 Dec;16(6):475-93. doi: 10.1007/s40257-015-0158-0. PMID: 26481651

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

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
Mycoplasma pneumoniae-induced rash and mucositis as a syndrome distinct from Stevens-Johnson syndrome and erythema multiforme: a systematic review.
Canavan TN, Mathes EF, Frieden I, Shinkai K
J Am Acad Dermatol 2015 Feb;72(2):239-45. doi: 10.1016/j.jaad.2014.06.026. PMID: 25592340
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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