SJS
MCID: STV001
MIFTS: 56

Stevens-Johnson Syndrome (SJS) malady

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

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NIH Rare Diseases:42 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 based summary: 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 and toxic epidermal necrolysis are two forms of a life-threatening skin... more...

Description from OMIM:46 608579

Aliases & Classifications for Stevens-Johnson Syndrome

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Stevens-Johnson Syndrome, Aliases & Descriptions:

Name: Stevens-Johnson Syndrome 30 8 42 46 10 44 62
Erythema Multiforme Major 42 62
 
Schwartz-Jampel Syndrome 62
Sjs 42


Classifications:

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


External Ids:

Disease Ontology8 DOID:0050426
MeSH34 D013262

Related Diseases for Stevens-Johnson Syndrome

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Diseases related to Stevens-Johnson Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 144)
idRelated DiseaseScoreTop Affiliating Genes
1toxic epidermal necrolysis31.8HSPG2, FAS, FASLG, GZMB
2myeloma30.9FASLG, FAS, HLA-B
3hepatitis30.8GZMB, FASLG, FAS
4systemic lupus erythematosus30.7HSPG2, FAS, FASLG
5lymphopenia30.6FAS, FASLG
6b-cell lymphomas30.5GZMB, FAS, HLA-B
7t-cell leukemia30.4HLA-B, FAS, FASLG
8leukemia30.1HLA-B, FAS, FASLG, GZMB
9rheumatoid arthritis30.0HSPG2, FAS, FASLG, GZMB
10sjogren's syndrome29.9HSPG2, FAS, FASLG, GZMB
11erythema multiforme11.1
12pneumonia10.9
13cicatricial pemphigoid10.7
14ocular cicatricial pemphigoid10.7
15burns10.6
16lupus erythematosus10.6
17multiple myeloma10.5
18uveitis10.5HLA-B
19bronchiolitis obliterans10.5
20bronchiolitis10.5
21cataract10.4
22neutropenia10.4
23conjunctival pigmentation10.4
24conjunctivitis10.4
25dermatitis10.4
26herpes simplex10.4
27hypersensitivity syndrome, carbamazepine-induced10.4
28acute generalized exanthematous pustulosis10.4
29t-cell large granular lymphocyte leukemia10.3FASLG, GZMB
30adult t-cell leukemia10.2
31arthritis10.2
32crohn's disease10.2
33diffuse large b-cell lymphoma10.2
34hemangioma10.2
35hematopoietic stem cell transplantation10.2
36hiv/aids10.2
37keratopathy10.2
38liver cirrhosis10.2
39renal cell carcinoma10.2
40skin disease10.2
41thrombocytopenia10.2
42vulvovaginitis10.2
43orbital cyst10.2
44neuroleptic malignant syndrome10.2
45dacryocystocele10.2
46folliculitis10.2
47aniridia10.2
48visceral leishmaniasis10.2
49bronchopneumonia10.2
50parotitis10.2

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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Clinical features from OMIM:

608579

Drugs & Therapeutics for Stevens-Johnson Syndrome

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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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MalaCards organs/tissues related to Stevens-Johnson Syndrome:

32
Skin, Eye, T cells, Liver, Colon, Lung, Salivary gland, Testes, Neutrophil, Bronchial epithelium, Monocytes, B cells

Animal Models for Stevens-Johnson Syndrome or affiliated genes

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MGI Mouse Phenotypes related to Stevens-Johnson Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107718.5HSPG2, TGM1, FAS, FASLG
2MP:00053978.0HSPG2, FAS, FASLG, GZMB, HLA-B
3MP:00053877.8FASLG, FAS, HSPG2, HLA-B, GZMB
4MP:00107687.7GZMB, HLA-B, HSPG2, TGM1, FAS, FASLG

Publications for Stevens-Johnson Syndrome

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Articles related to Stevens-Johnson Syndrome:

(show top 50)    (show all 551)
idTitleAuthorsYear
1
Late onset imatinib-induced Stevens-Johnson syndrome. (24399835)
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
Stevens-Johnson Syndrome associated with mogamulizumab treatment of adult T-cell leukemia / lymphoma. (23360455)
2013
5
Nevirapine and/or co-trimoxazole induced Stevens Johnson syndrome in HIV infected patient--a case report. (23656450)
2013
6
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
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
HLA-A*0206 with TLR3 polymorphisms exerts more than additive effects in Stevens-Johnson syndrome with severe ocular surface complications. (22912896)
2012
11
Stevens-Johnson syndrome induced by levetiracetam. (23036769)
2012
12
First case of mirtazepine-induced Stevens-Johnson syndrome from India. (23112435)
2012
13
Stevens-Johnson syndrome induced by acetazolamide. (21342230)
2011
14
Stevens Johnson Syndrome in a patient undergoing gynaecological brachytherapy: An association or an incident? (24376967)
2011
15
Rituxan is not associated with Stevens Johnson Syndrome. (21525404)
2011
16
Retrospective analysis of stevens-johnson syndrome and toxic epidermal necrolysis over a period of 10 years. (21572786)
2011
17
Stevens-Johnson syndrome after mycoplasma pneumoniae infection. (18614318)
2011
18
HLA-B*1502 genotyping in two Chinese patients with phenytoin-induced Stevens-Johnson syndrome. (21216202)
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
Stevens-Johnson syndrome in a boy with macrolide-resistant Mycoplasma pneumoniae pneumonia. (21536614)
2011
23
The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. (22014021)
2011
24
Association between prostaglandin E receptor 3 polymorphisms and Stevens-Johnson syndrome identified by means of a genome-wide association study. (20947153)
2010
25
Stevens-Johnson syndrome following occupational exposure to carbamate insecticide. (20175856)
2010
26
A case of bupropion-induced Stevens-Johnson syndrome with acute psoriatic exacerbation. (20684153)
2010
27
Early gynaecological assessment following Stevens-Johnson syndrome/toxic epidermal necrolysis. (21126137)
2010
28
Management of acute Stevens-Johnson syndrome and toxic epidermal necrolysis utilizing amniotic membrane and topical corticosteroids. (20005508)
2010
29
Erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis in lenalidomide-treated patients. (19047275)
2009
30
Stevens-Johnson syndrome and toxic epidermal necrolysis: consequence of treatment of an emerging pathogen. (19687711)
2009
31
The role of systemic corticosteroid therapy in erythema multiforme major and stevens-johnson syndrome: a review of past and current opinions. (20729944)
2009
32
An update on Stevens-Johnson syndrome and toxic epidermal necrolysis in children. (19474732)
2009
33
Oxcarbazepine-induced Stevens-Johnson syndrome: a case report. (19321411)
2009
34
Topical treatment protocol for Stevens-Johnson syndrome and toxic epidermal necrolysis: a brief report. (19752660)
2009
35
Vision-related function after scleral lens fitting in ocular complications of Stevens-Johnson syndrome and toxic epidermal necrolysis. (19837383)
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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Clinvar genetic disease variations for Stevens-Johnson Syndrome:

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 Stevens-Johnson Syndrome

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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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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 pathway37
9.6FASLG, GZMB
2
Show member pathways
Immune response IL 12 induced IFN gamma production60
9.6FASLG, GZMB
39.5FAS, FASLG
49.5FASLG, FAS
5
Show member pathways
TRAIL signaling pathway37
9.5FAS, FASLG
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
9.2HLA-B, FAS, FASLG
10
Show member pathways
FAS pathway and Stress induction of HSP regulation37
Apoptosis and survival FAS signaling cascades60
Caspase cascade in apoptosis37
9.1FASLG, FAS, GZMB
11
Show member pathways
Apoptosis Modulation and Signaling37
Apoptosis37
9.1FAS, GZMB, FASLG
12
Show member pathways
9.1GZMB, FAS, FASLG
139.1FASLG, GZMB, FAS
14
Show member pathways
9.1FAS, GZMB, FASLG
159.1FAS, FASLG, GZMB
168.9FAS, FASLG, HSPG2
17
Show member pathways
Toll-like receptor signaling pathway37
Regulation of toll-like receptor signaling pathway37
8.9FAS, HSPG2, FASLG
18
Show member pathways
Immune response CD16 signaling in NK cells60
8.7HLA-B, GZMB, FAS, FASLG
19
Show member pathways
Proteasome Degradation37
Immune response Antigen presentation by MHC class I60
8.7FAS, HLA-B, GZMB, FASLG
20
Show member pathways
8.7HLA-B, FAS, FASLG, GZMB

Compounds for genes affiliated with Stevens-Johnson Syndrome

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Compounds related to Stevens-Johnson Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 63)
idCompoundScoreTop Affiliating Genes
1chromium4410.0FASLG, GZMB
2(glc)4449.9FASLG, FAS
3atpo28 4410.9FASLG, FAS
4teniposide44 1110.9FASLG, FAS
52-chlorodeoxyadenosine449.8FASLG, FAS
6dutp449.7FAS, FASLG
73-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide449.7FASLG, FAS
8gold449.7FASLG, HLA-B, GZMB
9tacrolimus44 50 1111.6HLA-B, FASLG, GZMB
10depsipeptide449.6FASLG, FAS
11suberoylanilide hydroxamic acid449.6FAS, FASLG
12ethanol44 50 24 1112.6HLA-B, FASLG, GZMB
135-aza-2deoxycytidine449.5FAS, FASLG, HLA-B
14concanamycin a44 6110.5GZMB, FASLG, FAS
15ribonucleic acid449.5GZMB, FAS, HLA-B
16ivig449.5FAS, GZMB, FASLG
17fludarabine44 50 1111.5FAS, FASLG
18cytarabine44 50 1111.5GZMB, FASLG, FAS
19agarose449.5FASLG, FAS, GZMB
20edelfosine44 6110.5FAS, HSPG2
21polysaccharide449.5HLA-B, FAS, FASLG
22etoposide44 50 61 1112.4FASLG, FAS, GZMB
23rapamycin449.4GZMB, FASLG, FAS
24c2ceramide449.3FASLG, HSPG2, FAS
25rottlerin449.3HSPG2, FAS, FASLG
26ascorbic acid44 2410.2FASLG, HSPG2, FAS
27rantes449.2GZMB, FAS, FASLG, HLA-B
28indomethacin44 28 61 1112.2HSPG2, FAS, FASLG
29arginine449.2HLA-B, TGM1, GZMB, FAS
30paraffin449.2FASLG, GZMB, FAS, HLA-B
31cyclosporin a44 28 6111.2HLA-B, FAS, FASLG, GZMB
32lactate449.2HLA-B, FAS, GZMB, FASLG
33aspartate449.2GZMB, FASLG, HLA-B, FAS
34oligonucleotide449.1HLA-B, FASLG, GZMB, FAS
35pd 98,059449.1HSPG2, FASLG, FAS
36wortmannin449.1FASLG, FAS, HSPG2
37h2o2448.9HSPG2, HLA-B, FASLG, FAS
38phosphatidylserine44 28 1110.9HSPG2, GZMB, FAS, FASLG
39ly294002448.9FASLG, HSPG2, FAS, GZMB
40genistein44 28 61 2 24 1113.9HSPG2, FAS, FASLG
41heparin44 28 24 1111.9GZMB, HSPG2, FASLG, FAS
42glutamine448.9HSPG2, TGM1, FAS
43vegf448.8HSPG2, GZMB, FASLG, FAS
44nitric oxide44 24 1110.8FASLG, FAS, TGM1, GZMB, HLA-B
45pge2448.8HLA-B, FASLG, HSPG2
46dexamethasone44 50 28 1111.6HLA-B, GZMB, FAS, FASLG, HSPG2
47calcium44 50 24 1111.5GZMB, FASLG, TGM1, HLA-B, HSPG2
48serine448.5HLA-B, HSPG2, GZMB, FASLG, FAS
49tyrosine448.5FAS, HLA-B, FASLG, HSPG2, GZMB
50retinoic acid44 249.2FAS, FASLG, TGM1, HSPG2, HLA-B, GZMB

GO Terms for genes affiliated with Stevens-Johnson Syndrome

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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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3CDC
13ExPASy
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22GTR
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24HMDB
25ICD10
26ICD10 via Orphanet
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29KEGG
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