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Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome

MedGen UID:
783034
•Concept ID:
C3661878
•
Disease or Syndrome
Synonyms: SJS/TEN overlap syndrome; Stevens Johnson syndrome AND toxic epidermal necrolysis overlap; Stevens-Johnson syndrome - toxic epidermal necrolysis overlap; Stevens-Johnson/TEN overlap syndrome; Stevens-Johnson/toxic epidermal necrolysis overlap syndrome
SNOMED CT: Stevens Johnson syndrome AND toxic epidermal necrolysis overlap (124911000119100); Stevens-Johnson syndrome - toxic epidermal necrolysis overlap (124911000119100)
 
Monarch Initiative: MONDO:0044739
Orphanet: ORPHA506784

Definition

Severe mucocutaneous reactions; skin detachment of 10 to 30 percent of body surface area most commonly triggered by medications, characterized by extensive necrosis and detachment of the epidermis. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVStevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome

Professional guidelines

PubMed

Management of Drug-Induced Epidermal Necrolysis (DEN) in Pediatric Patients: Moving from Drug-Induced Stevens-Johnson Syndrome, Overlap and Toxic Epidermal Necrolysis to a Single Unifying Diagnosis of DEN.
Ramien ML, Mansour D, Shear NH
Paediatr Drugs 2022 Jul;24(4):307-319. Epub 2022 Jun 9 doi: 10.1007/s40272-022-00515-0. PMID: 35676614

Recent clinical studies

Etiology

Incidence, outcomes, and resource use in children with Stevens-Johnson syndrome and toxic epidermal necrolysis.
Antoon JW, Goldman JL, Lee B, Schwartz A
Pediatr Dermatol 2018 Mar;35(2):182-187. Epub 2018 Jan 9 doi: 10.1111/pde.13383. PMID: 29315761
Stevens-Johnson syndrome and toxic epidermal necrolysis at the University Hospital of the West Indies, Jamaica.
East-Innis AD, Thompson DS
West Indian Med J 2013 Sep;62(7):589-92. doi: 10.7727/wimj.2013.031. PMID: 24831894
Toll-like receptor 9 polymorphism in patients with erythema multiforme, Stevens Johnson syndrome and Stevens Johnson syndrome/toxic epidermal necrolysis overlap syndrome.
Turan H, Bulbul Baskan E, Yakut T, Karkucak M, Tunali S, Saricaoglu H
Bratisl Lek Listy 2011;112(5):260-3. PMID: 21682079

Diagnosis

Stevens-Johnson syndrome/toxic epidermal necrolysis: treatment with low-dose corticosteroids, vitamin C and thiamine.
Middendorf MM, Busaileh AZ, Babakhani A, Marik PE
BMJ Case Rep 2019 Nov 2;12(11) doi: 10.1136/bcr-2019-230538. PMID: 31678920Free PMC Article
Incidence, outcomes, and resource use in children with Stevens-Johnson syndrome and toxic epidermal necrolysis.
Antoon JW, Goldman JL, Lee B, Schwartz A
Pediatr Dermatol 2018 Mar;35(2):182-187. Epub 2018 Jan 9 doi: 10.1111/pde.13383. PMID: 29315761
Stevens-Johnson syndrome and toxic epidermal necrolysis at the University Hospital of the West Indies, Jamaica.
East-Innis AD, Thompson DS
West Indian Med J 2013 Sep;62(7):589-92. doi: 10.7727/wimj.2013.031. PMID: 24831894
Toll-like receptor 9 polymorphism in patients with erythema multiforme, Stevens Johnson syndrome and Stevens Johnson syndrome/toxic epidermal necrolysis overlap syndrome.
Turan H, Bulbul Baskan E, Yakut T, Karkucak M, Tunali S, Saricaoglu H
Bratisl Lek Listy 2011;112(5):260-3. PMID: 21682079

Therapy

Stevens-Johnson syndrome/toxic epidermal necrolysis: treatment with low-dose corticosteroids, vitamin C and thiamine.
Middendorf MM, Busaileh AZ, Babakhani A, Marik PE
BMJ Case Rep 2019 Nov 2;12(11) doi: 10.1136/bcr-2019-230538. PMID: 31678920Free PMC Article
Severe Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome-beyond skin involvement.
Milheiro Silva T, Farela Neves J, Casimiro A, Varandas L, Gouveia C
Pediatr Dermatol 2018 Jan;35(1):e17-e19. Epub 2017 Nov 21 doi: 10.1111/pde.13328. PMID: 29159901
Paracetamol induced Stevens-Johnson syndrome--toxic epidermal necrolysis overlap syndrome.
Biswal S, Sahoo SS
Int J Dermatol 2014 Aug;53(8):1042-4. Epub 2013 Oct 29 doi: 10.1111/ijd.12355. PMID: 24673330
Stevens-Johnson syndrome and toxic epidermal necrolysis at the University Hospital of the West Indies, Jamaica.
East-Innis AD, Thompson DS
West Indian Med J 2013 Sep;62(7):589-92. doi: 10.7727/wimj.2013.031. PMID: 24831894

Prognosis

Incidence, outcomes, and resource use in children with Stevens-Johnson syndrome and toxic epidermal necrolysis.
Antoon JW, Goldman JL, Lee B, Schwartz A
Pediatr Dermatol 2018 Mar;35(2):182-187. Epub 2018 Jan 9 doi: 10.1111/pde.13383. PMID: 29315761
Paracetamol induced Stevens-Johnson syndrome--toxic epidermal necrolysis overlap syndrome.
Biswal S, Sahoo SS
Int J Dermatol 2014 Aug;53(8):1042-4. Epub 2013 Oct 29 doi: 10.1111/ijd.12355. PMID: 24673330

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