SJS
MCID: STV001
MIFTS: 55

Stevens-Johnson Syndrome (SJS) malady

Rare diseases, Skin diseases categories
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Summaries for Stevens-Johnson Syndrome

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Sources:
43NIH Rare Diseases, 65Wikipedia, 47OMIM, 33MalaCards
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NIH Rare Diseases:43 Stevens-johnson syndrome (sjs), also called erythema multiforme major, is a limited form of toxic epidermal necrolysis. this disorder affects the skin, mucous membranes and eyes. stevens-johnson syndrome occurs twice as often in men as women, and most cases appear in children and young adults under 30, although it can develop in people at any age. it is an emergency medical condition that usually requires hospitalization. treatment focuses on eliminating the underlying cause, controlling symptoms and minimizing complications. last updated: 11/22/2010

MalaCards: Stevens-Johnson Syndrome, also known as erythema multiforme major, is related to toxic epidermal necrolysis and myeloma. An important gene associated with Stevens-Johnson Syndrome is HLA-B (major histocompatibility complex, class I, B), and among its related pathways are Downstream signaling in naive CD8+ T cells and IL12-mediated signaling events. The compounds chromium and (glc)4 have been mentioned in the context of this disorder. Affiliated tissues include skin, eye and t cells, and related mouse phenotypes are integument and hematopoietic system.

Wikipedia:65 Stevens?Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are two forms of a life-threatening... more...

Description from OMIM:47 608579

Aliases & Classifications for Stevens-Johnson Syndrome

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Sources:
8Disease Ontology, 43NIH Rare Diseases, 47OMIM, 10DISEASES, 45Novoseek, 31LifeMap Discovery™, 62UMLS, 35MeSH
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Classifications:

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


Aliases & Descriptions:

stevens-johnson syndrome 8 43 47 10 45 31 62
erythema multiforme major 43 62
schwartz-jampel syndrome 62
sjs 43


External Ids:

Disease Ontology8 DOID:0050426
MeSH35 D013262

Related Diseases for Stevens-Johnson Syndrome

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Stevens-Johnson Syndrome:



Diseases related to stevens-johnson syndrome

Symptoms for Stevens-Johnson Syndrome

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47OMIM
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Clinical features from OMIM:

608579

Drugs & Therapeutics for Stevens-Johnson Syndrome

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 31LifeMap Discovery™, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Stevens-Johnson Syndrome

Search NIH Clinical Center for Stevens-Johnson Syndrome

Cell-based therapeutics:


LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine
Read about Stevens-Johnson Syndrome cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Stevens-Johnson Syndrome:
Cultivated corneal epithelial stem cells for treatment of ocular surface damage
Embryonic/Adult Cultured Cells Related to Stevens-Johnson Syndrome:
Limbal corneal epithelial stem cells, PMID: 23055668

Genetic Tests for Stevens-Johnson Syndrome

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Anatomical Context for Stevens-Johnson Syndrome

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33MalaCards
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MalaCards organs/tissues related to Stevens-Johnson Syndrome:

33
Skin, Eye, T cells, Liver, Salivary gland, Colon, Lung, Testes, Monocytes, Bronchial epithelium, B cells

Animal Models for Stevens-Johnson Syndrome or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Stevens-Johnson Syndrome:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107718.5FASLG, FAS, TGM1, HSPG2
2MP:00053978.0HLA-B, HSPG2, FAS, FASLG, GZMB
3MP:00053877.8HLA-B, HSPG2, FAS, FASLG, GZMB
4MP:00107687.7GZMB, FASLG, FAS, TGM1, HSPG2, HLA-B

Publications for Stevens-Johnson Syndrome

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Sources:
52PubMed
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Articles related to Stevens-Johnson Syndrome:

(show top 50)    (show all 528)
idTitleAuthorsYear
1
Late onset imatinib-induced Stevens-Johnson syndrome. (24399835)
2014
2
Stevens-Johnson syndrome and toxic epidermal necrolysis in children. (24028417)
2013
3
Stevens-Johnson Syndrome associated with mogamulizumab treatment of adult T-cell leukemia / lymphoma. (23360455)
2013
4
Nevirapine and/or co-trimoxazole induced Stevens Johnson syndrome in HIV infected patient--a case report. (23656450)
2013
5
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
6
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
7
Comprehensive survival analysis of a cohort of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis. (23389396)
2013
8
Genetic association of IFN-I^ +874T/A polymorphism in Mexican patients with drug-induced Stevens-Johnson syndrome/toxic epidermal necrolysis. (23224615)
2013
9
HLA-A*0206 with TLR3 polymorphisms exerts more than additive effects in Stevens-Johnson syndrome with severe ocular surface complications. (22912896)
2012
10
Stevens-Johnson syndrome induced by levetiracetam. (23036769)
2012
11
First case of mirtazepine-induced Stevens-Johnson syndrome from India. (23112435)
2012
12
Stevens-Johnson syndrome induced by acetazolamide. (21342230)
2011
13
Stevens Johnson Syndrome in a patient undergoing gynaecological brachytherapy: An association or an incident? (24376967)
2011
14
Rituxan is not associated with Stevens Johnson Syndrome. (21525404)
2011
15
Retrospective analysis of stevens-johnson syndrome and toxic epidermal necrolysis over a period of 10 years. (21572786)
2011
16
Stevens-Johnson syndrome after mycoplasma pneumoniae infection. (18614318)
2011
17
HLA-B*1502 genotyping in two Chinese patients with phenytoin-induced Stevens-Johnson syndrome. (21216202)
2011
18
Stevens-Johnson syndrome and toxic epidermal necrolysis in children: a review of the experience with paediatric patients in a university hospital. (21198948)
2011
19
Bilateral microbial keratitis in highly active antiretroviral therapy-induced Stevens-Johnson syndrome and toxic epidermal necrolysis: a case series. (21834698)
2011
20
A systematic review of treatment of drug-induced Stevens-Johnson syndrome and toxic epidermal necrolysis in children. (21467603)
2011
21
Stevens-Johnson syndrome in a boy with macrolide-resistant Mycoplasma pneumoniae pneumonia. (21536614)
2011
22
The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. (22014021)
2011
23
Association between prostaglandin E receptor 3 polymorphisms and Stevens-Johnson syndrome identified by means of a genome-wide association study. (20947153)
2010
24
Stevens-Johnson syndrome following occupational exposure to carbamate insecticide. (20175856)
2010
25
A case of bupropion-induced Stevens-Johnson syndrome with acute psoriatic exacerbation. (20684153)
2010
26
Early gynaecological assessment following Stevens-Johnson syndrome/toxic epidermal necrolysis. (21126137)
2010
27
Management of acute Stevens-Johnson syndrome and toxic epidermal necrolysis utilizing amniotic membrane and topical corticosteroids. (20005508)
2010
28
Erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis in lenalidomide-treated patients. (19047275)
2009
29
Stevens-Johnson syndrome and toxic epidermal necrolysis: consequence of treatment of an emerging pathogen. (19687711)
2009
30
The role of systemic corticosteroid therapy in erythema multiforme major and stevens-johnson syndrome: a review of past and current opinions. (20729944)
2009
31
An update on Stevens-Johnson syndrome and toxic epidermal necrolysis in children. (19474732)
2009
32
Oxcarbazepine-induced Stevens-Johnson syndrome: a case report. (19321411)
2009
33
Topical treatment protocol for Stevens-Johnson syndrome and toxic epidermal necrolysis: a brief report. (19752660)
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
Stevens-Johnson syndrome-induced facial scar treated by the fractional photothermolysis system. (18330799)
2008
38
HLA-B locus in Japanese patients with anti-epileptics and allopurinol-related Stevens-Johnson syndrome and toxic epidermal necrolysis. (19018717)
2008
39
A case of Stevens-Johnson syndrome possibly induced by amifostine during radiotherapy. (18338505)
2007
40
Lamotrigine-induced Stevens-Johnson syndrome. (17244876)
2007
41
Toll-like receptor 3 gene polymorphisms in Japanese patients with Stevens-Johnson syndrome. (17314152)
2007
42
Intravenous immunoglobulins for the management of Stevens-Johnson syndrome with minimal skin manifestations. (17008995)
2007
43
The timing of intravenous immunoglobulin therapy in Stevens-Johnson syndrome and toxic epidermal necrolysis. (16045716)
2005
44
Stevens - Johnson syndrome due to nevirapine. (16615847)
2005
45
Risk of Stevens-Johnson syndrome and toxic epidermal necrolysis in new users of antiepileptics. (15824335)
2005
46
Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. (15875523)
2005
47
Intravenous immunoglobulin prophylaxis for recurrent Stevens-Johnson syndrome. (14726889)
2004
48
Stevens-Johnson syndrome associated with ciprofloxacin: a review of adverse cutaneous events reported in Sweden as associated with this drug. (14576649)
2003
49
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
50
Correction of entropion from Stevens-Johnson syndrome: use of nasal septum and mucosa for severely cicatrized eyelid entropion. (779733)
1976

Variations for Stevens-Johnson Syndrome

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Expression for genes affiliated with Stevens-Johnson Syndrome

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Sources:
2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Stevens-Johnson Syndrome

Search GEO for disease gene expression data for Stevens-Johnson Syndrome.

Pathways for genes affiliated with Stevens-Johnson Syndrome

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Sources:
50PathCards, 38NCBI BioSystems Database, 60Thomson Reuters, 30KEGG, 55Reactome, 54R&D Systems, 5Cell Signaling Technology, 53QIAGEN
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Pathways related to Stevens-Johnson Syndrome according to GeneCards/GeneDecks:

(show all 20)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
Ras signaling in the CD4+ TCR pathway38
9.6GZMB, FASLG
2
Show member pathways
Immune response IL 12 induced IFN gamma production60
9.6GZMB, FASLG
39.5FASLG, FAS
49.5FAS, FASLG
5
Show member pathways
TRAIL signaling pathway38
9.5FASLG, FAS
6
Show member pathways
9.5FASLG, FAS
7
Show member pathways
Apoptosis and survival Apoptotic TNF family pathways60
9.5FASLG, 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
9.5FASLG, FAS
9
Show member pathways
9.2FASLG, FAS, HLA-B
10
Show member pathways
FAS pathway and Stress induction of HSP regulation38
Apoptosis and survival FAS signaling cascades60
Caspase cascade in apoptosis38
9.1FAS, FASLG, GZMB
11
Show member pathways
Apoptosis Modulation and Signaling38
Apoptosis38
9.1GZMB, FASLG, FAS
12
Show member pathways
9.1FAS, FASLG, GZMB
139.1FAS, FASLG, GZMB
14
Show member pathways
9.1GZMB, FASLG, FAS
159.1GZMB, FASLG, FAS
168.9FASLG, FAS, HSPG2
17
Show member pathways
Toll-like receptor signaling pathway38
Regulation of toll-like receptor signaling pathway38
8.9HSPG2, FAS, FASLG
18
Show member pathways
Immune response CD16 signaling in NK cells60
8.7HLA-B, FAS, FASLG, GZMB
19
Show member pathways
Proteasome Degradation38
Immune response Antigen presentation by MHC class I60
8.7HLA-B, FAS, FASLG, GZMB
20
Show member pathways
8.7GZMB, FASLG, FAS, HLA-B

Compounds for genes affiliated with Stevens-Johnson Syndrome

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Sources:
45Novoseek, 29IUPHAR, 11DrugBank, 51PharmGKB, 24HMDB, 61Tocris Bioscience, 3BitterDB
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Compounds related to Stevens-Johnson Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 63)
idCompoundScoreTop Affiliating Genes
1chromium4510.0FASLG, GZMB
2(glc)4459.9FASLG, FAS
3atpo29 4510.9FAS, FASLG
4teniposide45 1110.9FAS, FASLG
52-chlorodeoxyadenosine459.8FASLG, FAS
6dutp459.7FAS, FASLG
73-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide459.7FAS, FASLG
8gold459.7GZMB, FASLG, HLA-B
9tacrolimus45 51 1111.6GZMB, FASLG, HLA-B
10depsipeptide459.6FASLG, FAS
11suberoylanilide hydroxamic acid459.6FASLG, FAS
12ethanol45 51 24 1112.6GZMB, FASLG, HLA-B
135-aza-2deoxycytidine459.5FASLG, FAS, HLA-B
14concanamycin a45 6110.5GZMB, FASLG, FAS
15ribonucleic acid459.5HLA-B, FAS, GZMB
16ivig459.5GZMB, FASLG, FAS
17fludarabine45 51 1111.5FASLG, FAS
18cytarabine45 51 1111.5FAS, FASLG, GZMB
19agarose459.5GZMB, FASLG, FAS
20edelfosine45 6110.5FAS, HSPG2
21polysaccharide459.5HLA-B, FAS, FASLG
22etoposide45 51 61 1112.4GZMB, FASLG, FAS
23rapamycin459.4FAS, FASLG, GZMB
24rottlerin459.3HSPG2, FAS, FASLG
25c2ceramide459.3HSPG2, FAS, FASLG
26ascorbic acid45 2410.2FASLG, FAS, HSPG2
27rantes459.2GZMB, FASLG, FAS, HLA-B
28indomethacin45 29 61 1112.2FASLG, FAS, HSPG2
29arginine459.2GZMB, FAS, TGM1, HLA-B
30paraffin459.2HLA-B, FAS, FASLG, GZMB
31cyclosporin a45 29 6111.2HLA-B, FAS, FASLG, GZMB
32lactate459.2HLA-B, FAS, FASLG, GZMB
33aspartate459.2HLA-B, FAS, FASLG, GZMB
34oligonucleotide459.1HLA-B, FAS, FASLG, GZMB
35pd 98,059459.1HSPG2, FAS, FASLG
36wortmannin459.1FASLG, FAS, HSPG2
37h2o2458.9HLA-B, HSPG2, FAS, FASLG
38phosphatidylserine45 29 1110.9HSPG2, FAS, FASLG, GZMB
39ly294002458.9HSPG2, FAS, FASLG, GZMB
40genistein45 29 61 3 24 1113.9HSPG2, FAS, FASLG
41heparin45 29 24 1111.9GZMB, FASLG, FAS, HSPG2
42glutamine458.9FAS, TGM1, HSPG2
43vegf458.8GZMB, FASLG, FAS, HSPG2
44nitric oxide45 24 1110.8HLA-B, TGM1, FAS, FASLG, GZMB
45pge2458.8FASLG, HSPG2, HLA-B
46dexamethasone45 51 29 1111.6HLA-B, HSPG2, FAS, FASLG, GZMB
47calcium45 51 24 1111.5HLA-B, HSPG2, TGM1, FASLG, GZMB
48serine458.5HLA-B, HSPG2, FAS, FASLG, GZMB
49tyrosine458.5HLA-B, HSPG2, FAS, FASLG, GZMB
50retinoic acid45 249.2GZMB, HLA-B, HSPG2, TGM1, FAS, FASLG

GO Terms for genes affiliated with Stevens-Johnson Syndrome

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16Gene Ontology
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Cellular components related to Stevens-Johnson Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1lysosomal lumenGO:0432029.4FASLG, HSPG2
2extracellular vesicular exosomeGO:0700627.9FASLG, FAS, TGM1, HSPG2, HLA-B

Biological processes related to Stevens-Johnson Syndrome according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1inflammatory cell apoptotic processGO:0069259.7FASLG, FAS
2necroptotic signaling pathwayGO:0975279.6FASLG, FAS
3regulation of extrinsic apoptotic signaling pathway in absence of ligandGO:20012399.6FASLG, FAS
4extrinsic apoptotic signaling pathwayGO:0971919.6FAS, FASLG
5extrinsic apoptotic signaling pathway via death domain receptorsGO:0086259.5FAS, FASLG
6defense responseGO:0069529.2HCP5, HLA-B
7activation of cysteine-type endopeptidase activity involved in apoptotic processGO:0069199.2FASLG, FAS
8apoptotic processGO:0069159.1GZMB, FASLG, FAS

Products for genes affiliated with Stevens-Johnson Syndrome

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Sources for Stevens-Johnson Syndrome

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet