MCID: PRN049
MIFTS: 35

Paraneoplastic Pemphigus malady

Skin diseases category

Summaries for Paraneoplastic Pemphigus

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63Wikipedia, 32MalaCards
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Wikipedia:63 Paraneoplastic pemphigus is an autoimmune disorder that is almost invariably linked to an underlying... more...

MalaCards: Paraneoplastic Pemphigus is related to pemphigus and bronchiolitis obliterans, and has symptoms including vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachmentand enanthema/aphtosa/aphta/leukoplakia. An important gene associated with Paraneoplastic Pemphigus is DSP (desmoplakin), and among its related pathways are Adhesion and Type I hemidesmosome assembly. The compounds dr-10 and asulam have been mentioned in the context of this disorder. Affiliated tissues include skin, and related mouse phenotype integument.

Aliases & Classifications for Paraneoplastic Pemphigus

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48Orphanet, 60UMLS, 26ICD10 via Orphanet, 61UMLS via Orphanet
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Classifications:

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


Characteristics (Orphanet epidemiological data):

48
paraneoplastic pemphigus:
Inheritance: Sporadic; Prevalence: <1/1000000; Age of onset: Variable; Age of death: Any age


Aliases & Descriptions:

paraneoplastic pemphigus 48 60


External Ids:

ICD10 via Orphanet26 L10.8
UMLS via Orphanet61 C1112570

Related Diseases for Paraneoplastic Pemphigus

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17GeneCards, 18GeneDecks
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Diseases related to Paraneoplastic Pemphigus via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 172)
idRelated DiseaseScoreTop Affiliating Genes
1pemphigus31.8JUP, PPL, DSP, DSG3, DST, DSG1
2bronchiolitis obliterans30.9PPL
3sarcoma30.9JUP, PPL, DSP, DSG1, EVPL
4follicular dendritic cell sarcoma30.7PPL
5thymoma30.7EVPL, DSP, PPL
6lichen planus30.7DST, DSG3, COL17A1
7leukemia30.7HLA-C, HLA-B, HLA-DRB1, HLA-A, BLZF1, CD79A
8myasthenia gravis30.5HLA-DQB1, HLA-B
9lupus erythematosus30.3CD79A
10conjunctivitis30.3CD79A
11dermatitis30.3DSG3, DSG1, CD79A
12bullous pemphigoid30.3COL17A1, PPL, DSP, DST, EVPL
13hepatocellular carcinoma30.3JUP, HLA-DRB1, HLA-A
14systemic lupus erythematosus30.2HLA-DQB1, HLA-DRB1, HLA-A, C6orf25, BLZF1, CD79A
15squamous cell carcinoma30.2HLA-A, HLA-B, PPL, JUP, COL17A1, DSP
16tetanus30.0CD79A, HLA-B
17inflammatory bowel disease30.0JUP, HLA-DQB1, HLA-B, DSG2
18hepatitis c30.0HLA-A, HLA-DRB1, HLA-C
19skin disease30.0COL17A1, HLA-C, DSP, DSG3, DST, DSG1
20melanoma30.0JUP, HLA-C, HLA-B, HLA-DRB1, HLA-A, DST
21hepatitis b30.0HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A, CD79A
22bronchiolitis10.6
23dendritic cell sarcoma10.6
24hodgkin's lymphoma10.5
25chronic lymphocytic leukemia10.4
26respiratory failure10.4
27non-hodgkin lymphoma10.3
28stomatitis10.3
29lichen disease10.2
30mediastinitis10.2
31graft versus host disease10.1
32burns10.1
33lymphoid leukemia10.1
34acanthosis nigricans10.1
35idiopathic pulmonary fibrosis10.1
36encapsulated thymoma10.1
37fibrous histiocytoma10.1
38common variable immunodeficiency10.1
39interdigitating dendritic cell sarcoma10.1
40lung lymphoma10.1
41epithelioid leiomyosarcoma10.1
42bronchogenic carcinoma10.1
43anaplastic large cell lymphoma10.1
44diffuse large b-cell lymphoma10.1
45retroperitoneal lymphoma10.1
46chronic conjunctivitis10.1
47dendritic cell thymoma10.1
48endometrial carcinoma10.1
49esophagitis10.1
50fibrosarcoma10.1

Graphical network of the top 20 diseases related to Paraneoplastic Pemphigus:



Diseases related to paraneoplastic pemphigus

Clinical Features for Paraneoplastic Pemphigus

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48Orphanet
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Symptoms:

48
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • enanthema/aphtosa/aphta/leukoplakia

Drugs & Therapeutics for Paraneoplastic Pemphigus

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

Search CenterWatch for Paraneoplastic Pemphigus

Drug clinical trials:

Search ClinicalTrials for Paraneoplastic Pemphigus

Search NIH Clinical Center for Paraneoplastic Pemphigus

Search CenterWatch for Paraneoplastic Pemphigus

Genetic Tests for Paraneoplastic Pemphigus

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Anatomical Context for Paraneoplastic Pemphigus

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32MalaCards
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MalaCards organs/tissues related to Paraneoplastic Pemphigus:

32
Skin

Animal Models for Paraneoplastic Pemphigus or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Paraneoplastic Pemphigus:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107718.9EVPL, DST, DSG3, JUP, COL17A1

Publications for Paraneoplastic Pemphigus

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Genetic Variations for Paraneoplastic Pemphigus

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Expression for genes affiliated with Paraneoplastic Pemphigus

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Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Paraneoplastic Pemphigus

Search GEO for disease gene expression data for Paraneoplastic Pemphigus.

Pathways for genes affiliated with Paraneoplastic Pemphigus

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Sources:
4Cell Signaling Technology, 53Reactome, 29KEGG, 37NCBI BioSystems Database, 12EMD Millipore, 51QIAGEN
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Pathways related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

(show all 26)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8PLEC, DSP, JUP
29.7PLEC, DST, COL17A1
39.7HLA-DQB1, HLA-DRB1, DSG1
4
Hide members
9.6DSG2, DSC2, DSP, JUP
5
Immune response NFAT in immune response
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9.6CD79A, HLA-DRB1, HLA-DQB1
69.5PLEC, DST, DSP, COL17A1
7
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9.5COL17A1, JUP, DST, PLEC
8
Immune response IFN alpha/beta signaling pathway
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9.3HLA-A, HLA-B, HLA-C
9
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9.3HLA-A, HLA-B, HLA-C
10
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9.3HLA-A, HLA-B, HLA-C
119.3HLA-A, HLA-B, HLA-C
129.3HLA-C, HLA-B, HLA-A
139.2DSG2, DSG1, DSG3, DSP
149.1PPL, DSP, DST, PLEC, EVPL
15
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9.0HLA-C, HLA-B, HLA-A, CD79A
16
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9.0DSP, DSG3, DSG1, DSG2, PLEC
17
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8.7HLA-A, HLA-DRB1, HLA-B, HLA-DQB1, HLA-C
18
Hide members
8.7HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
198.7HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
208.7HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
21
Hide members
8.7HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
22
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8.7HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
238.7HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
248.7HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
25
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8.4HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A, CD79A
26
Hide members
7.8HLA-C, HLA-B, HLA-A, DSP, DSG3, DSG1

Compounds for genes affiliated with Paraneoplastic Pemphigus

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Sources:
44Novoseek, 11DrugBank, 24HMDB, 49PharmGKB, 28IUPHAR
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Compounds related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

(show all 14)
idCompoundScoreTop Affiliating Genes
1dr-104410.0HLA-A, HLA-DRB1
2asulam4410.0DSP, PPL
3pneumococcal449.8HLA-B, CD79A
4toxin a449.8DSG3, DSG1
5ivig449.5DSG3, DSG1, CD79A
6allergens449.5CD79A, BLZF1, HLA-DRB1
7sucrose44 11 2411.3COL17A1, JUP, DSP, DSG1, BLZF1
8oligonucleotide448.8JUP, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A, DST
9steroid448.8CD79A, BLZF1, DSP, HLA-B, COL17A1
10acetylcholine44 49 28 11 2412.7CD79A, BLZF1, DSG1, DSG3
11retinoic acid44 249.7CD79A, BLZF1, DSG1, DSP, HLA-B, JUP
12serine448.3COL17A1, JUP, HLA-B, DSP, DSG3, DSG1
13calcium44 49 11 2410.2DSP, C6orf25, JUP, COL17A1, DSG3, DST
14tyrosine446.5BLZF1, COL17A1, JUP, HLA-DQB1, HLA-B, HLA-DRB1

GO Terms for genes affiliated with Paraneoplastic Pemphigus

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16Gene Ontology
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Cellular components related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

(show all 12)
idNameGO IDScoreTop Affiliating Genes
1MHC class II protein complexGO:0426139.9HLA-DRB1, HLA-DQB1
2hemidesmosomeGO:0300569.7PLEC, DST, JUP, COL17A1
3cell-cell junctionGO:0059119.6COL17A1, JUP, DSG1, DSG2
4intermediate filamentGO:0058829.5DST, DSP, JUP
5MHC class I protein complexGO:0426129.5HLA-C, HLA-B, HLA-A
6phagocytic vesicle membraneGO:0306709.3HLA-A, HLA-B, HLA-C
7early endosome membraneGO:0319019.2HLA-C, HLA-B, HLA-A
8integral to lumenal side of endoplasmic reticulum membraneGO:0715569.0HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
9ER to Golgi transport vesicle membraneGO:0125078.9HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
10Golgi membraneGO:0001398.3BLZF1, HLA-A, HLA-DRB1, HLA-B, HLA-DQB1, HLA-C
11desmosomeGO:0300578.3EVPL, JUP, PPL, DSP, DSG3, DSC2
12plasma membraneGO:0058865.1HLA-DRB1, HLA-B, HLA-DQB1, HLA-C, PPL, JUP

Biological processes related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

(show all 23)
idNameGO IDScoreTop Affiliating Genes
1immunoglobulin production involved in immunoglobulin mediated immune responseGO:00238110.1HLA-DRB1, HLA-DQB1
2humoral immune response mediated by circulating immunoglobulinGO:00245510.1HLA-DRB1, HLA-DQB1
3hemidesmosome assemblyGO:03158110.0PLEC, DST, COL17A1
4bundle of His cell to Purkinje myocyte communicationGO:08606910.0DSG2, DSC2, DSP, JUP
5epidermis developmentGO:00854410.0COL17A1, DSP, EVPL
6regulation of ventricular cardiac muscle cell action potentialGO:08600510.0DSG2, DSC2, DSP, JUP
7regulation of heart rate by cardiac conductionGO:0860919.9JUP, DSP, DSC2, DSG2
8cell junction assemblyGO:0343299.9COL17A1, JUP, PLEC
9cell-cell adhesionGO:0163379.8DSG1, DSP, JUP
10detection of bacteriumGO:0160459.7HLA-B, HLA-DRB1, HLA-A
11antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independentGO:0024809.7HLA-A, HLA-B, HLA-C
12positive regulation of T cell mediated cytotoxicityGO:0019169.6HLA-A, HLA-B, HLA-C
13type I interferon-mediated signaling pathwayGO:0603379.6HLA-C, HLA-B, HLA-A
14antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependentGO:0024799.5HLA-C, HLA-B, HLA-A
15homophilic cell adhesionGO:0071569.5DSG3, DSC2, DSG1, DSG2
16antigen processing and presentation of exogenous peptide antigen via MHC class IGO:0425909.4HLA-A, HLA-B, HLA-C
17cellular component disassembly involved in execution phase of apoptosisGO:0069219.3DSP, DSG3, DSG1, DSG2, PLEC
18regulation of immune responseGO:0507769.2HLA-C, HLA-B, HLA-A
19interferon-gamma-mediated signaling pathwayGO:0603339.1HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
20antigen processing and presentation of peptide antigen via MHC class IGO:0024749.0HLA-A, HLA-B, HLA-C
21cytokine-mediated signaling pathwayGO:0192219.0HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
22immune responseGO:0069559.0HLA-C, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A
23apoptotic processGO:0069159.0PLEC, DSG2, DSG1, DSG3, DSP

Molecular functions related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1MHC class II receptor activityGO:0323959.9HLA-DRB1, HLA-DQB1
2peptide antigen bindingGO:0426058.7HLA-A, HLA-DRB1, HLA-B, HLA-C

Products for genes affiliated with Paraneoplastic Pemphigus

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Paraneoplastic Pemphigus

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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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet