TEN
MCID: TXC012
MIFTS: 51

Toxic Epidermal Necrolysis (TEN) malady

Rare diseases, Skin diseases categories

Summaries for Toxic Epidermal Necrolysis

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66Wikipedia, 48OMIM, 34MalaCards
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Wikipedia:66 Toxic epidermal necrolysis (TEN), also known as Lyell\'s syndrome, is a rare, life-threatening skin... more...

MalaCards: Toxic Epidermal Necrolysis, also known as staphylococcal scalded skin syndrome, is related to stevens-johnson syndrome and graft versus host disease. An important gene associated with Toxic Epidermal Necrolysis is HLA-B (major histocompatibility complex, class I, B), and among its related pathways are IL12-mediated signaling events and Downstream signaling in naive CD8+ T cells. The compounds (glc)4 and atpo have been mentioned in the context of this disorder. Affiliated tissues include skin, liver and lung, and related mouse phenotypes are immune system and hematopoietic system.

Description from OMIM:48 608579

Aliases & Classifications for Toxic Epidermal Necrolysis

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Sources:
44NIH Rare Diseases, 63UMLS, 48OMIM, 46Novoseek, 26ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases


Aliases & Descriptions:

toxic epidermal necrolysis 44 48 46 63
staphylococcal scalded skin syndrome 44 63
mucocutaneous exfoliative disease 44
sick sinus syndrome 63
ssss 44
ten 44


External Ids:

ICD1026 L51.2

Related Diseases for Toxic Epidermal Necrolysis

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18GeneCards, 19GeneDecks
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Diseases related to Toxic Epidermal Necrolysis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 134)
idRelated DiseaseScoreTop Affiliating Genes
1stevens-johnson syndrome31.9GZMB, PSORS1C1, FASLG, FAS, HSPG2, HLA-B
2graft versus host disease30.8GZMB, FASLG, FAS
3systemic lupus erythematosus30.8HSPG2, FAS, FASLG, KLRC2
4lung cancer30.7HLA-B, HSPG2, FAS, GZMB
5myeloma30.6HLA-B, FAS, FASLG
6psoriasis30.5GZMB, HLA-B
7lymphopenia30.5FASLG, FAS
8aplastic anemia30.4GZMB, FASLG, FAS
9hepatitis30.4FASLG, FAS, GZMB
10b-cell lymphomas30.4HLA-B, FAS, GZMB
11colon cancer30.4FASLG, GZMB, FAS
12melanoma30.3FAS, HLA-B, FASLG, HSPG2, GZMB
13rheumatoid arthritis30.3HSPG2, FASLG, KLRC2, GZMB, FAS
14erythema multiforme11.0
15burns10.8
16lupus erythematosus10.8
17lyell syndrome10.8
18acute generalized exanthematous pustulosis10.8
19bullous impetigo10.7
20cutaneous lupus erythematosus10.7
21ritter's disease10.6
22dermatitis10.6
23pemphigus10.5
24contact dermatitis10.5
25parotitis10.4
26ecthyma10.4
27epidermolysis bullosa10.4
28multiple myeloma10.4
29pneumonia10.4
30skin disease10.4
31tuberculosis10.4
32hypersensitivity syndrome, carbamazepine-induced10.4
33sepsis10.4
34leukopenia10.4
35subacute cutaneous lupus erythematosus10.4
36paraneoplastic pemphigus10.4
37molluscum contagiosum10.3
38congenital ichthyosiform erythroderma10.3
39ophthalmia neonatorum10.3
40pyomyositis10.3
41septic arthritis10.3
42netherton syndrome10.3
43diffuse cutaneous mastocytosis10.3
44toxic shock syndrome10.3
45cutaneous mastocytosis10.3
46miliaria10.3
47mastocytosis10.3
48generalized pustular psoriasis10.3
49rubella10.3
50vulvovaginitis10.3

Graphical network of the top 20 diseases related to Toxic Epidermal Necrolysis:



Diseases related to toxic epidermal necrolysis

Symptoms for Toxic Epidermal Necrolysis

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48OMIM
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Clinical features from OMIM:

608579

Drugs & Therapeutics for Toxic Epidermal Necrolysis

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

Search CenterWatch for Toxic Epidermal Necrolysis

Drug clinical trials:

Search ClinicalTrials for Toxic Epidermal Necrolysis

Search NIH Clinical Center for Toxic Epidermal Necrolysis

Search CenterWatch for Toxic Epidermal Necrolysis

Genetic Tests for Toxic Epidermal Necrolysis

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Anatomical Context for Toxic Epidermal Necrolysis

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34MalaCards
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MalaCards organs/tissues related to Toxic Epidermal Necrolysis:

34
Skin, Liver, Lung, T cells, Brain, Colon, Testes, Monocytes, B cells

Animal Models for Toxic Epidermal Necrolysis or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Toxic Epidermal Necrolysis:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053878.4HSPG2, FAS, FASLG, GZMB, HLA-B
2MP:00053978.1GZMB, FASLG, FAS, HSPG2, HLA-B

Publications for Toxic Epidermal Necrolysis

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53PubMed
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Articles related to Toxic Epidermal Necrolysis:

(show top 50)    (show all 565)
idTitleAuthorsYear
1
Stevens-Johnson syndrome and toxic epidermal necrolysis in children. (24028417)
2013
2
Relationship between the HLA-B*1502 allele and carbamazepine-induced Stevens-Johnson syndrome and toxic epidermal necrolysis: a systematic review and meta-analysis. (23884208)
2013
3
Toxic epidermal necrolysis: the year in review. (23799330)
2013
4
Hospital management and clinical factors associated with ophthalmic involvement in Toxic Epidermal Necrolysis. (24286611)
2013
5
Association between the HLA-B*15:02 allele and carbamazepine-induced Stevens-Johnson syndrome/toxic epidermal necrolysis in Han individuals of northeastern China. (24399721)
2013
6
Comprehensive survival analysis of a cohort of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. (23389396)
2013
7
Genetic association of IFN-I^ +874T/A polymorphism in Mexican patients with drug-induced Stevens-Johnson syndrome/toxic epidermal necrolysis. (23224615)
2013
8
Proteomic kinetic analysis of blister fluid and serum in a patient with drug-induced toxic epidermal necrolysis. A comparison with skin immunohistochemistry. (23373548)
2012
9
Toxic epidermal necrolysis-like subacute cutaneous lupus erythematosus. (23157783)
2012
10
Rhabdomyolysis as a potential complication of carbamazepine-induced toxic epidermal necrolysis. (22728955)
2012
11
Toxic epidermal necrolysis after pemetrexed and cisplatin for non-small cell lung cancer in a patient with sharp syndrome. (23207626)
2012
12
Toxic epidermal necrolysis associated with Mycoplasma bovis in calves. (22562103)
2012
13
Immunological mechanisms of epidermal damage in toxic epidermal necrolysis. (22688730)
2012
14
The management of toxic epidermal necrolysis. (22881464)
2012
15
Sodium nitroprusside and toxic epidermal necrolysis. (23156856)
2012
16
Retrospective analysis of stevens-johnson syndrome and toxic epidermal necrolysis over a period of 10 years. (21572786)
2011
17
Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a review of the experience with paediatric patients in a university hospital. (21198948)
2011
18
Bilateral microbial keratitis in highly active antiretroviral therapy-induced Stevens-Johnson syndrome and toxic epidermal necrolysis: a case series. (21834698)
2011
19
A systematic review of treatment of drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children. (21467603)
2011
20
The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. (22014021)
2011
21
Toxic epidermal necrolysis in pregnancy: case report and review of the literature. (20671561)
2010
22
Levofloxacin-induced toxic epidermal necrolysis treated with intravenous immunoglobulin. (20838711)
2010
23
Early gynaecological assessment following Stevens-Johnson syndrome/toxic epidermal necrolysis. (21126137)
2010
24
Management of acute Stevens-Johnson syndrome and toxic epidermal necrolysis utilizing amniotic membrane and topical corticosteroids. (20005508)
2010
25
Vaginal stenosis after toxic epidermal necrolysis. (20885170)
2010
26
Asymptomatic hyperamylasemia and hyperlipasemia in pediatric patients with toxic epidermal necrolysis. (20182382)
2010
27
Erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis in lenalidomide-treated patients. (19047275)
2009
28
Toxic epidermal necrolysis in a patient with primary myelofibrosis receiving thalidomide therapy. (19052692)
2009
29
Inflammatory epidermolysis bullosa acquisita mimicking toxic epidermal necrolysis and dermatitis herpetiformis. (19663858)
2009
30
Stevens-Johnson syndrome and toxic epidermal necrolysis: consequence of treatment of an emerging pathogen. (19687711)
2009
31
An update on Stevens-Johnson syndrome and toxic epidermal necrolysis in children. (19474732)
2009
32
Topical treatment protocol for Stevens-Johnson syndrome and toxic epidermal necrolysis: a brief report. (19752660)
2009
33
Interleukin 8 as a chemoattractant for neutrophil granulocytes in toxic epidermal necrolysis lesions. (19175789)
2009
34
Vision-related function after scleral lens fitting in ocular complications of Stevens-Johnson syndrome and toxic epidermal necrolysis. (19837383)
2009
35
Strong association between HLA-B*5801 and allopurinol-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in a Thai population. (19696695)
2009
36
Serum interleukin-13 levels are increased in patients with Stevens-Johnson syndrome/ toxic epidermal necrolysis but not in those with erythema multiforme. (17941938)
2008
37
Immunohistochemical evaluation of toxic epidermal necrolysis treated with human intravenous immunoglobulin. (18833079)
2008
38
HLA-B locus in Japanese patients with anti-epileptics and allopurinol-related Stevens-Johnson syndrome and toxic epidermal necrolysis. (19018717)
2008
39
Epidermal interleukin-8 and its receptor CXCR2 in drug-induced toxic epidermal necrolysis. (17714530)
2007
40
Management of scalp toxic epidermal necrolysis and cranial osteomyelitis with serratus anterior myocutaneous pedicle flap: a case report. (17438486)
2007
41
Toxic epidermal necrolysis in a child successfully treated with cyclosporin A and methylprednisolone. (17875095)
2007
42
The timing of intravenous immunoglobulin therapy in Stevens-Johnson syndrome and toxic epidermal necrolysis. (16045716)
2005
43
Risk of Stevens-Johnson syndrome and toxic epidermal necrolysis in new users of antiepileptics. (15824335)
2005
44
Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. (15875523)
2005
45
Correlations between clinical patterns and causes of erythema multiforme majus, Stevens-Johnson syndrome, and toxic epidermal necrolysis: results of an international prospective study. (12164739)
2002
46
Acute generalized exanthematous pustulosis mimicking toxic epidermal necrolysis. (11679003)
2001
47
Immunoregulatory effector cells in drug-induced toxic epidermal necrolysis. (11048976)
2000
48
Gelatinases in drug-induced toxic epidermal necrolysis. (9726031)
1998
49
Toxic epidermal necrolysis (Lyell syndrome). A case report. (6192286)
1983
50
Haemolysin and enzyme patterns of coagulase-positive staphylococci isolated from toxic epidermal necrolysis, Ritter's disease and impetigo contagiosa. (4252396)
1969

Variations for Toxic Epidermal Necrolysis

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1 National Center for Biotechnology Information (Clinvar)
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Clinvar genetic disease variations for Toxic Epidermal Necrolysis:

1
id Gene Name Type Significance SNP ID Assembly Location
1SCN5ANM_000335.4(SCN5A): c.3890C> T (p.Pro1297Leu)single nucleotide variantPathogenicrs28937319GRCh37Chr 3, 38603976: 38603976

Expression for genes affiliated with Toxic Epidermal Necrolysis

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Toxic Epidermal Necrolysis

Search GEO for disease gene expression data for Toxic Epidermal Necrolysis.

Pathways for genes affiliated with Toxic Epidermal Necrolysis

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Sources:
51PathCards, 39NCBI BioSystems Database, 61Thomson Reuters, 31KEGG, 55R&D Systems, 56Reactome, 5Cell Signaling Technology, 54QIAGEN
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Pathways related to Toxic Epidermal Necrolysis according to GeneCards/GeneDecks:

(show all 20)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
Immune response IL 12 induced IFN gamma production61
9.7GZMB, FASLG
2
Show member pathways
Ras signaling in the CD4+ TCR pathway39
9.7GZMB, FASLG
39.6FAS, FASLG
49.6FASLG, FAS
5
Show member pathways
TRAIL signaling pathway39
9.6FASLG, FAS
6
Show member pathways
Apoptosis and survival Apoptotic TNF family pathways61
9.6FASLG, FAS
7
Show member pathways
9.6FASLG, FAS
8
Show member pathways
Transcription NF kB signaling pathway61
Immune response Toll like receptor TLR ligands and common TLR signaling pathway leading to cell proinflammatory response61
9.6FASLG, FAS
9
Show member pathways
9.3FASLG, FAS, HLA-B
10
Show member pathways
FAS pathway and Stress induction of HSP regulation39
Apoptosis and survival FAS signaling cascades61
Caspase cascade in apoptosis39
9.2FAS, FASLG, GZMB
11
Show member pathways
9.2GZMB, FASLG, FAS
12
Show member pathways
Apoptosis Modulation and Signaling39
Apoptosis39
9.2GZMB, FASLG, FAS
139.2GZMB, FASLG, FAS
14
Show member pathways
9.2FAS, FASLG, GZMB
159.2FAS, FASLG, GZMB
16
Show member pathways
Toll-like receptor signaling pathway39
Regulation of toll-like receptor signaling pathway39
9.1HSPG2, FAS, FASLG
179.1FASLG, FAS, HSPG2
18
Show member pathways
Proteasome Degradation39
Immune response Antigen presentation by MHC class I61
8.9HLA-B, FAS, FASLG, GZMB
19
Show member pathways
8.4HLA-B, FAS, FASLG, KLRC2, GZMB
20
Show member pathways
Immune response CD16 signaling in NK cells61
8.4GZMB, KLRC2, FASLG, FAS, HLA-B

Compounds for genes affiliated with Toxic Epidermal Necrolysis

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Sources:
46Novoseek, 30IUPHAR, 12DrugBank, 52PharmGKB, 62Tocris Bioscience, 25HMDB, 3BitterDB
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Compounds related to Toxic Epidermal Necrolysis according to GeneCards/GeneDecks:

(show top 50)    (show all 59)
idCompoundScoreTop Affiliating Genes
1(glc)44610.1FASLG, FAS
2atpo30 4611.0FAS, FASLG
3chromium4610.0FASLG, GZMB
4teniposide46 1211.0FAS, FASLG
52-chlorodeoxyadenosine469.9FASLG, FAS
63-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide469.9FASLG, FAS
7dutp469.9FASLG, FAS
8depsipeptide469.8FASLG, FAS
9suberoylanilide hydroxamic acid469.8FASLG, FAS
10gold469.8GZMB, FASLG, HLA-B
11tacrolimus46 52 1211.7GZMB, FASLG, HLA-B
12edelfosine46 6210.7HSPG2, FAS
135-aza-2deoxycytidine469.7FASLG, FAS, HLA-B
14concanamycin a46 6210.7GZMB, FASLG, FAS
15ivig469.7GZMB, FASLG, FAS
16ethanol46 52 25 1212.7GZMB, FASLG, HLA-B
17fludarabine46 52 1211.6FASLG, FAS
18cytarabine46 52 1211.6FAS, FASLG, GZMB
19ifn-alpha469.6FASLG, FAS
20agarose469.6GZMB, FASLG, FAS
21polysaccharide469.6HLA-B, FAS, FASLG
22ribonucleic acid469.6HLA-B, FAS, GZMB
23etoposide46 52 62 1212.6GZMB, FASLG, FAS
24rottlerin469.6HSPG2, FAS, FASLG
25c2ceramide469.6HSPG2, FAS, FASLG
26rapamycin469.5FAS, FASLG, GZMB
27tetrapeptide469.5GZMB, FAS
28ascorbic acid46 2510.5FASLG, FAS, HSPG2
29indomethacin46 30 62 1212.4FASLG, FAS, HSPG2
30pd 98,059469.4HSPG2, FAS, FASLG
31wortmannin469.4FASLG, FAS, HSPG2
32calyculin a46 62 1211.3FASLG, FAS
33rantes469.3HLA-B, FAS, FASLG, GZMB
34paraffin469.3GZMB, FASLG, FAS, HLA-B
35cyclosporin a46 30 6211.3HLA-B, FAS, FASLG, GZMB
36lactate469.3HLA-B, FAS, FASLG, GZMB
37aspartate469.3HLA-B, FAS, FASLG, GZMB
38pge2469.3HLA-B, HSPG2, FASLG
39oligonucleotide469.3HLA-B, FAS, FASLG, GZMB
40nitric oxide46 25 1211.2GZMB, FASLG, FAS, HLA-B
41genistein46 30 62 3 25 1214.2FASLG, FAS, HSPG2
42h2o2469.2HLA-B, HSPG2, FAS, FASLG
43phosphatidylserine46 30 1211.2GZMB, FASLG, FAS, HSPG2
44ly294002469.2HSPG2, FAS, FASLG, GZMB
45heparin46 30 25 1212.1GZMB, FASLG, FAS, HSPG2
46vegf469.1GZMB, FASLG, FAS, HSPG2
47dexamethasone46 52 30 1211.8HLA-B, HSPG2, FAS, FASLG, GZMB
48retinoic acid46 259.8HLA-B, HSPG2, FAS, FASLG, GZMB
49serine468.8HLA-B, HSPG2, FAS, FASLG, GZMB
50tyrosine468.7HLA-B, HSPG2, FAS, FASLG, GZMB

GO Terms for genes affiliated with Toxic Epidermal Necrolysis

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17Gene Ontology
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Cellular components related to Toxic Epidermal Necrolysis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1lysosomal lumenGO:0432029.3FASLG, HSPG2
2extracellular vesicular exosomeGO:0700628.8FASLG, FAS, HSPG2, HLA-B

Biological processes related to Toxic Epidermal Necrolysis according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1inflammatory cell apoptotic processGO:0069259.8FASLG, FAS
2necroptotic signaling pathwayGO:0975279.8FASLG, FAS
3regulation of extrinsic apoptotic signaling pathway in absence of ligandGO:20012399.8FASLG, FAS
4extrinsic apoptotic signaling pathwayGO:0971919.7FAS, FASLG
5extrinsic apoptotic signaling pathway via death domain receptorsGO:0086259.6FAS, FASLG
6defense responseGO:0069529.4HCP5, HLA-B
7activation of cysteine-type endopeptidase activity involved in apoptotic processGO:0069199.3FASLG, FAS
8apoptotic processGO:0069159.2GZMB, FASLG, FAS

Products for genes affiliated with Toxic Epidermal Necrolysis

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Sources for Toxic Epidermal Necrolysis

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet