TEN
MCID: TXC012
MIFTS: 53

Toxic Epidermal Necrolysis (TEN) malady

Rare diseases, Skin diseases categories
Download this MalaCard

Summaries for Toxic Epidermal Necrolysis

About this section


Fully expand this MalaCard
Wikipedia:65 Toxic epidermal necrolysis (TEN), also known as Lyell\'s syndrome, is a rare, life-threatening skin... more...

MalaCards based summary: 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:46 608579

Aliases & Classifications for Toxic Epidermal Necrolysis

About this section
Sources:
62UMLS, 42NIH Rare Diseases, 46OMIM, 44Novoseek
See all sources

Toxic Epidermal Necrolysis, Aliases & Descriptions:

Name: Toxic Epidermal Necrolysis 42 46 44 62
Staphylococcal Scalded Skin Syndrome 42
Mucocutaneous Exfoliative Disease 42
Scalded Skin Syndrome 62
 
Sick Sinus Syndrome 62
Ssss 42
Ten 42


Classifications:

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


Related Diseases for Toxic Epidermal Necrolysis

About this section

Diseases in the Toxic Epidermal Necrolysis family:

Susceptibility to Toxic Epidermal Necrolysis Due to Allopurinol Treatment Susceptibility to Toxic Epidermal Necrolysis Due to Carbamazepine Treatment
Susceptibility to Toxic Epidermal Necrolysis Due to Phenytoin Treatment Susceptibility to Toxic Epidermal Necrolysis Due to Phosphenytoin Treatment

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

(show top 50)    (show all 138)
idRelated DiseaseScoreTop Affiliating Genes
1stevens-johnson syndrome31.3HLA-B, HSPG2, FAS, FASLG, PSORS1C1, GZMB
2graft versus host disease31.0GZMB, FASLG, FAS
3myeloma30.9HLA-B, FAS, FASLG
4psoriasis30.8GZMB, HLA-B
5lymphopenia30.7FAS, FASLG
6systemic lupus erythematosus30.7HSPG2, FAS, FASLG, KLRC2
7aplastic anemia30.6GZMB, FASLG, FAS
8hepatitis30.6GZMB, FASLG, FAS
9lung cancer30.5HLA-B, HSPG2, FAS, GZMB
10b-cell lymphomas30.5HLA-B, FAS, GZMB
11melanoma30.0HLA-B, HSPG2, FAS, FASLG, GZMB
12rheumatoid arthritis30.0HSPG2, FAS, FASLG, KLRC2, GZMB
13erythema multiforme11.0
14burns10.8
15lupus erythematosus10.8
16acute generalized exanthematous pustulosis10.8
17lyell syndrome10.8
18bullous impetigo10.8
19cutaneous lupus erythematosus10.7
20ritter's disease10.6
21dermatitis10.6
22multiple myeloma10.5
23pemphigus10.5
24contact dermatitis10.5
25hypersensitivity syndrome, carbamazepine-induced10.5
26susceptibility to toxic epidermal necrolysis due to phosphenytoin treatment10.5
27uveitis10.5HLA-B
28skin disease10.4
29ecthyma10.4
30parotitis10.4
31eosinophilia10.4
32epidermolysis bullosa10.4
33pneumonia10.4
34tuberculosis10.4
35sepsis10.4
36leukopenia10.4
37subacute cutaneous lupus erythematosus10.4
38susceptibility to toxic epidermal necrolysis due to allopurinol treatment10.4
39susceptibility to toxic epidermal necrolysis due to carbamazepine treatment10.4
40susceptibility to toxic epidermal necrolysis due to phenytoin treatment10.4
41paraneoplastic pemphigus10.4
42toxic shock syndrome10.3
43congenital ichthyosiform erythroderma10.3
44pyomyositis10.3
45septic arthritis10.3
46ophthalmia neonatorum10.3
47netherton syndrome10.3
48molluscum contagiosum10.3
49miliaria10.3
50generalized pustular psoriasis10.3

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



Diseases related to toxic epidermal necrolysis

Symptoms for Toxic Epidermal Necrolysis

About this section


Clinical features from OMIM:

608579

Drugs & Therapeutics for Toxic Epidermal Necrolysis

About this section

Drug clinical trials:

Search ClinicalTrials for Toxic Epidermal Necrolysis

Search NIH Clinical Center for Toxic Epidermal Necrolysis

Genetic Tests for Toxic Epidermal Necrolysis

About this section

Anatomical Context for Toxic Epidermal Necrolysis

About this section

MalaCards organs/tissues related to Toxic Epidermal Necrolysis:

32
Skin, Liver, Lung, T cells, Brain, Neutrophil, Colon, Testes, Monocytes, B cells

Animal Models for Toxic Epidermal Necrolysis or affiliated genes

About this section

MGI Mouse Phenotypes related to Toxic Epidermal Necrolysis:

36
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

About this section

Articles related to Toxic Epidermal Necrolysis:

(show top 50)    (show all 589)
idTitleAuthorsYear
1
Severe flucloxacillin-induced acute generalized exanthematous pustulosis (AGEP), with toxic epidermal necrolysis (TEN)-like features: does overlap between AGEP and TEN exist? Clinical report and review of the literature. (24888401)
2014
2
Use of intravenous immunoglobulin for Stevens-Johnson syndrome and toxic epidermal necrolysis in children: Report of two cases secondary to anticonvulsants. (25183634)
2014
3
Stevens-Johnson syndrome and toxic epidermal necrolysis in children. (24028417)
2013
4
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
5
Toxic epidermal necrolysis: the year in review. (23799330)
2013
6
Hospital management and clinical factors associated with ophthalmic involvement in Toxic Epidermal Necrolysis. (24286611)
2013
7
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
8
Comprehensive survival analysis of a cohort of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. (23389396)
2013
9
Genetic association of IFN-I^ +874T/A polymorphism in Mexican patients with drug-induced Stevens-Johnson syndrome/toxic epidermal necrolysis. (23224615)
2013
10
Proteomic kinetic analysis of blister fluid and serum in a patient with drug-induced toxic epidermal necrolysis. A comparison with skin immunohistochemistry. (23373548)
2012
11
Toxic epidermal necrolysis-like subacute cutaneous lupus erythematosus. (23157783)
2012
12
Toxic epidermal necrolysis caused by fluconazole in a patient with human immunodeficiency virus infection. (23129968)
2012
13
Rhabdomyolysis as a potential complication of carbamazepine-induced toxic epidermal necrolysis. (22728955)
2012
14
Toxic epidermal necrolysis after pemetrexed and cisplatin for non-small cell lung cancer in a patient with sharp syndrome. (23207626)
2012
15
Toxic epidermal necrolysis associated with Mycoplasma bovis in calves. (22562103)
2012
16
Immunological mechanisms of epidermal damage in toxic epidermal necrolysis. (22688730)
2012
17
The management of toxic epidermal necrolysis. (22881464)
2012
18
Retrospective analysis of stevens-johnson syndrome and toxic epidermal necrolysis over a period of 10 years. (21572786)
2011
19
Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a review of the experience with paediatric patients in a university hospital. (21198948)
2011
20
Bilateral microbial keratitis in highly active antiretroviral therapy-induced Stevens-Johnson syndrome and toxic epidermal necrolysis: a case series. (21834698)
2011
21
A systematic review of treatment of drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children. (21467603)
2011
22
The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. (22014021)
2011
23
Toxic epidermal necrolysis in pregnancy: case report and review of the literature. (20671561)
2010
24
Levofloxacin-induced toxic epidermal necrolysis treated with intravenous immunoglobulin. (20838711)
2010
25
Early gynaecological assessment following Stevens-Johnson syndrome/toxic epidermal necrolysis. (21126137)
2010
26
Management of acute Stevens-Johnson syndrome and toxic epidermal necrolysis utilizing amniotic membrane and topical corticosteroids. (20005508)
2010
27
Vaginal stenosis after toxic epidermal necrolysis. (20885170)
2010
28
Asymptomatic hyperamylasemia and hyperlipasemia in pediatric patients with toxic epidermal necrolysis. (20182382)
2010
29
Erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis in lenalidomide-treated patients. (19047275)
2009
30
Toxic epidermal necrolysis in a patient with primary myelofibrosis receiving thalidomide therapy. (19052692)
2009
31
Inflammatory epidermolysis bullosa acquisita mimicking toxic epidermal necrolysis and dermatitis herpetiformis. (19663858)
2009
32
Stevens-Johnson syndrome and toxic epidermal necrolysis: consequence of treatment of an emerging pathogen. (19687711)
2009
33
An update on Stevens-Johnson syndrome and toxic epidermal necrolysis in children. (19474732)
2009
34
Topical treatment protocol for Stevens-Johnson syndrome and toxic epidermal necrolysis: a brief report. (19752660)
2009
35
Interleukin 8 as a chemoattractant for neutrophil granulocytes in toxic epidermal necrolysis lesions. (19175789)
2009
36
Vision-related function after scleral lens fitting in ocular complications of Stevens-Johnson syndrome and toxic epidermal necrolysis. (19837383)
2009
37
Serum interleukin-13 levels are increased in patients with Stevens-Johnson syndrome/ toxic epidermal necrolysis but not in those with erythema multiforme. (17941938)
2008
38
Immunohistochemical evaluation of toxic epidermal necrolysis treated with human intravenous immunoglobulin. (18833079)
2008
39
HLA-B locus in Japanese patients with anti-epileptics and allopurinol-related Stevens-Johnson syndrome and toxic epidermal necrolysis. (19018717)
2008
40
Epidermal interleukin-8 and its receptor CXCR2 in drug-induced toxic epidermal necrolysis. (17714530)
2007
41
Management of scalp toxic epidermal necrolysis and cranial osteomyelitis with serratus anterior myocutaneous pedicle flap: a case report. (17438486)
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

About this section

Clinvar genetic disease variations for Toxic Epidermal Necrolysis:

6
id Gene Name Type Significance SNP ID Assembly Location
1HLA-BHLA-B*15: 02undetermined variantrisk factor
2HLA-BHLA-B, HLA-B*5801undetermined variantrisk factor

Expression for genes affiliated with Toxic Epidermal Necrolysis

About this section
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

About this section

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 production60
9.7GZMB, FASLG
2
Show member pathways
Ras signaling in the CD4+ TCR pathway37
9.7GZMB, FASLG
39.6FAS, FASLG
49.6FASLG, FAS
5
Show member pathways
TRAIL signaling pathway37
9.6FASLG, FAS
6
Show member pathways
Apoptosis and survival Apoptotic TNF family pathways60
9.6FASLG, FAS
7
Show member pathways
9.6FASLG, FAS
8
Show member pathways
Transcription NF kB signaling pathway60
Immune response Toll like receptor TLR ligands and common TLR signaling pathway leading to cell proinflammatory response60
9.6FASLG, FAS
9
Show member pathways
9.3FASLG, FAS, HLA-B
10
Show member pathways
FAS pathway and Stress induction of HSP regulation37
Apoptosis and survival FAS signaling cascades60
Caspase cascade in apoptosis37
9.2FAS, FASLG, GZMB
11
Show member pathways
9.2GZMB, FASLG, FAS
12
Show member pathways
Apoptosis Modulation and Signaling37
Apoptosis37
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 pathway37
Regulation of toll-like receptor signaling pathway37
9.1HSPG2, FAS, FASLG
179.1FASLG, FAS, HSPG2
18
Show member pathways
Proteasome Degradation37
Immune response Antigen presentation by MHC class I60
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 cells60
8.4GZMB, KLRC2, FASLG, FAS, HLA-B

Compounds for genes affiliated with Toxic Epidermal Necrolysis

About this section

Compounds related to Toxic Epidermal Necrolysis according to GeneCards/GeneDecks:

(show top 50)    (show all 59)
idCompoundScoreTop Affiliating Genes
1(glc)44410.1FASLG, FAS
2atpo28 4411.0FAS, FASLG
3chromium4410.0FASLG, GZMB
4teniposide44 1111.0FAS, FASLG
52-chlorodeoxyadenosine449.9FASLG, FAS
63-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide449.9FASLG, FAS
7dutp449.9FASLG, FAS
8depsipeptide449.8FASLG, FAS
9suberoylanilide hydroxamic acid449.8FASLG, FAS
10gold449.8GZMB, FASLG, HLA-B
11tacrolimus44 50 1111.7GZMB, FASLG, HLA-B
12edelfosine44 6110.7HSPG2, FAS
135-aza-2deoxycytidine449.7FASLG, FAS, HLA-B
14concanamycin a44 6110.7GZMB, FASLG, FAS
15ivig449.7GZMB, FASLG, FAS
16ethanol44 50 24 1112.7GZMB, FASLG, HLA-B
17fludarabine44 50 1111.6FASLG, FAS
18cytarabine44 50 1111.6FAS, FASLG, GZMB
19ifn-alpha449.6FASLG, FAS
20agarose449.6GZMB, FASLG, FAS
21polysaccharide449.6HLA-B, FAS, FASLG
22ribonucleic acid449.6HLA-B, FAS, GZMB
23etoposide44 50 61 1112.6GZMB, FASLG, FAS
24rottlerin449.6HSPG2, FAS, FASLG
25c2ceramide449.6HSPG2, FAS, FASLG
26rapamycin449.5FAS, FASLG, GZMB
27tetrapeptide449.5GZMB, FAS
28ascorbic acid44 2410.5FASLG, FAS, HSPG2
29indomethacin44 28 61 1112.4FASLG, FAS, HSPG2
30pd 98,059449.4HSPG2, FAS, FASLG
31wortmannin449.4FASLG, FAS, HSPG2
32calyculin a44 61 1111.3FASLG, FAS
33rantes449.3HLA-B, FAS, FASLG, GZMB
34paraffin449.3GZMB, FASLG, FAS, HLA-B
35cyclosporin a44 28 6111.3HLA-B, FAS, FASLG, GZMB
36lactate449.3HLA-B, FAS, FASLG, GZMB
37aspartate449.3HLA-B, FAS, FASLG, GZMB
38pge2449.3HLA-B, HSPG2, FASLG
39oligonucleotide449.3HLA-B, FAS, FASLG, GZMB
40nitric oxide44 24 1111.2GZMB, FASLG, FAS, HLA-B
41genistein44 28 61 2 24 1114.2FASLG, FAS, HSPG2
42h2o2449.2HLA-B, HSPG2, FAS, FASLG
43phosphatidylserine44 28 1111.2GZMB, FASLG, FAS, HSPG2
44ly294002449.2HSPG2, FAS, FASLG, GZMB
45heparin44 28 24 1112.1GZMB, FASLG, FAS, HSPG2
46vegf449.1GZMB, FASLG, FAS, HSPG2
47dexamethasone44 50 28 1111.8HLA-B, HSPG2, FAS, FASLG, GZMB
48retinoic acid44 249.8HLA-B, HSPG2, FAS, FASLG, GZMB
49serine448.8HLA-B, HSPG2, FAS, FASLG, GZMB
50tyrosine448.7HLA-B, HSPG2, FAS, FASLG, GZMB

GO Terms for genes affiliated with Toxic Epidermal Necrolysis

About this section

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

About this section
  • Antibodies
  • Proteins
  • Lysates

Sources for Toxic Epidermal Necrolysis

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet