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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

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 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

Drug Hypersensitivity Reactions.
Wilkerson RG
Immunol Allergy Clin North Am 2023 Aug;43(3):473-489. doi: 10.1016/j.iac.2022.10.005. PMID: 37394254
Sulfonamide Hypersensitivity.
Chow TG, Khan DA
Clin Rev Allergy Immunol 2022 Jun;62(3):400-412. Epub 2021 Jul 1 doi: 10.1007/s12016-021-08872-3. PMID: 34212341
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
Bullous Drug Reactions.
Mockenhaupt M
Acta Derm Venereol 2020 Feb 12;100(5):adv00057. doi: 10.2340/00015555-3408. PMID: 32039459Free 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

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
A Review of Fixed Drug Eruption with a Special Focus on Generalized Bullous Fixed Drug Eruption.
Anderson HJ, Lee JB
Medicina (Kaunas) 2021 Sep 1;57(9) doi: 10.3390/medicina57090925. PMID: 34577848Free PMC Article
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
Bullous Drug Reactions.
Mockenhaupt M
Acta Derm Venereol 2020 Feb 12;100(5):adv00057. doi: 10.2340/00015555-3408. PMID: 32039459Free PMC Article

Therapy

Toxic Epidermal Necrolysis: A Review of Past and Present Therapeutic Approaches.
Singh N, Phillips M
Skin Therapy Lett 2022 Sep;27(5):7-13. PMID: 36469487
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
FR-Ciclosporin as a First-Line Treatment in Epidermal Necrolysis.
Morgado-sCarrasco D, Fustà-Novell X, Iranzo P
Actas Dermosifiliogr (Engl Ed) 2019 Sep;110(7):601-603. Epub 2019 Apr 7 doi: 10.1016/j.ad.2018.01.014. PMID: 30971332
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
Methotrexate-induced necrolysis.
Reed KM, Sober AJ
J Am Acad Dermatol 1983 May;8(5):677-9. doi: 10.1016/s0190-9622(83)70079-4. PMID: 6863626

Prognosis

Reactive infectious mucocutaneous eruption: Mycoplasma pneumoniae-induced rash and mucositis and other parainfectious eruptions.
Ramien ML
Clin Exp Dermatol 2021 Apr;46(3):420-429. Epub 2020 Sep 12 doi: 10.1111/ced.14404. PMID: 32918499
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
EMPACT syndrome.
Bilgili SG, Calka O, Karadag AS, Burakgazi AZ
Cutan Ocul Toxicol 2011 Dec;30(4):328-30. Epub 2011 Jun 16 doi: 10.3109/15569527.2011.588298. PMID: 21675930

Clinical prediction guides

Cutaneous Hypersensitivity Reactions to Immune Checkpoint Inhibitors.
Storgard R, Markova A
J Allergy Clin Immunol Pract 2024 May;12(5):1132-1136. Epub 2024 Mar 26 doi: 10.1016/j.jaip.2024.03.034. PMID: 38548170
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
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.
Castelain F, Humbert P
Curr Drug Saf 2012 Nov 1;7(5):332-8. doi: 10.2174/157488612805076516. PMID: 23441982
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

Ocular involvement in Mycoplasma induced rash and mucositis: A systematic review of the literature.
Haseeb A, Elhusseiny AM, ElSheikh RH, Tahboub MA, Kwan JT, Saeed HN
Ocul Surf 2023 Apr;28:1-10. Epub 2022 Nov 14 doi: 10.1016/j.jtos.2022.11.007. PMID: 36396020
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
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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