MCID: PRN049
MIFTS: 43

Paraneoplastic Pemphigus malady

Skin category

Summaries for Paraneoplastic Pemphigus

Sources:
64Wikipedia, 33MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
Wikipedia:64 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, tongue and lung, and related mouse phenotype integument.

Aliases & Classifications for Paraneoplastic Pemphigus

Sources:
49Orphanet, 61UMLS, 26ICD10 via Orphanet, 62UMLS via Orphanet
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
paraneoplastic pemphigus:
Inheritance: Sporadic; Prevalence: <1/1000000; Age of onset: Variable; Age of death: Any age


Aliases & Descriptions:

paraneoplastic pemphigus 49 61


External Ids:

ICD10 via Orphanet26 L10.8
UMLS via Orphanet62 C1112570

Related Diseases for Paraneoplastic Pemphigus

Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 167)
idRelated DiseaseScoreTop Affiliating Genes
1pemphigus31.9JUP, PPL, DSP, DSG3, DST, DSG1
2bronchiolitis obliterans30.9PPL
3pemphigus vulgaris30.9JUP, PPL, HLA-DRB1, DSP, DSG3, DST
4follicular dendritic cell sarcoma30.8PPL
5thymoma30.8EVPL, DSP, PPL
6lichen planus30.8DST, DSG3, COL17A1
7pemphigus foliaceus30.8DSG1, DSG2, CD79A, EVPL, DST, DSG3
8pemphigus vegetans30.5DSG1
9pemphigus erythematosus30.3DSP, DSG3
10bullous pemphigoid30.3COL17A1, PPL, DSP, DST, EVPL
11systemic lupus erythematosus30.3HLA-DQB1, HLA-DRB1, HLA-A, C6orf25, BLZF1, CD79A
12squamous cell carcinoma30.3HLA-A, HLA-B, PPL, JUP, COL17A1, DSP
13polymyositis30.1HLA-B
14tetanus30.1CD79A, HLA-B
15hepatitis c30.1HLA-A, HLA-DRB1, HLA-C
16bronchiolitis10.6
17castleman's disease10.5
18follicular lymphoma10.5
19stomatitis10.3
20lichen planus pemphigoides10.3
21inflammatory myofibroblastic tumor10.2
22toxic epidermal necrolysis10.2
23herpetiform pemphigus10.2
24graft versus host disease10.1
25lymphoid leukemia10.1
26acanthosis nigricans10.1
27idiopathic pulmonary fibrosis10.1
28encapsulated thymoma10.1
29fibrous histiocytoma10.1
30common variable immunodeficiency10.1
31interdigitating dendritic cell sarcoma10.1
32lung lymphoma10.1
33epithelioid leiomyosarcoma10.1
34bronchogenic carcinoma10.1
35anaplastic large cell lymphoma10.1
36diffuse large b-cell lymphoma10.1
37n syndrome10.1
38retroperitoneal lymphoma10.1
39hepatitis a10.1
40systemic mastocytosis10.1
41t-cell leukemia10.1
42vascular disease10.1
43angioimmunoblastic t-cell lymphoma10.1
44leukemia, t-cell, chronic10.1
45follicular dendritic cell tumor10.1
46retroperitoneal liposarcoma10.1
47lymphoblastic lymphoma10.1
48mastocytosis10.1
49esophageal squamous cell carcinoma10.1
50lymphoma, diffuse large cell10.1

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



Diseases related to paraneoplastic pemphigus

Clinical Features for Paraneoplastic Pemphigus

Sources:
49Orphanet
See all sources


Symptoms:

49
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • enanthema/aphtosa/aphta/leukoplakia

Drugs & Therapeutics for Paraneoplastic Pemphigus

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials
See all sources

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

Anatomical Context for Paraneoplastic Pemphigus

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Paraneoplastic Pemphigus:

33
Skin, Tongue, Lung, Bone marrow, Prostate, T cells, B lymphoblasts, B cells, Bronchial epithelium

Animal Models for Paraneoplastic Pemphigus or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Paraneoplastic Pemphigus:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107718.9EVPL, DST, DSG3, JUP, COL17A1

Publications for Paraneoplastic Pemphigus

Sources:
51PubMed
See all sources

Articles related to Paraneoplastic Pemphigus:

(show top 50)    (show all 273)
idTitleAuthorsYear
1
A case report of paraneoplastic pemphigus associated with esophageal squamous cell carcinoma. (23613673)
2013
2
Alemtuzumab is effective against severe chronic lymphocytic leukaemia-associated paraneoplastic pemphigus. (23517368)
2013
3
A skin rash and what lies beneath: paraneoplastic pemphigus, an atypical presentation of follicular cell lymphoma. (23658142)
2013
4
Case of paraneoplastic pemphigus with follicular lymphoma treated with rituximab. (23398274)
2013
5
Paraneoplastic pemphigus associated with chronic lymphocytic leukaemia: treatment with alemtuzumab. (22579369)
2012
6
Decline of disease activity and autoantibodies to desmoglein 3 and envoplakin by oral prednisolone in paraneoplastic pemphigus with benign thymoma. (22548836)
2012
7
Paraneoplastic pemphigus: a paraneoplastic autoimmune multiorgan syndrome or autoimmune multiorganopathy? (23316398)
2012
8
Paraneoplastic pemphigus with Castleman's disease and bronchiolitis obliterans. (22181577)
2011
9
Clinical images: Castleman disease and paraneoplastic pemphigus. (19884299)
2010
10
Paraneoplastic pemphigus with negative direct immunofluorescence in epidermis or mucosa but positive findings in adnexal structures. (18564282)
2009
11
Coexistence of paraneoplastic pemphigus and bullous pemphigoid. (19207671)
2009
12
Detection of anti-envoplakin and anti-periplakin autoantibodies by ELISA in patients with paraneoplastic pemphigus. (18820940)
2009
13
Development of ELISA for the specific determination of autoantibodies against envoplakin and periplakin in paraneoplastic pemphigus. (19737550)
2009
14
Detection and characterization of anti-envoplakin linker autoantibodies in paraneoplastic pemphigus using specific bead-based assay. (18790674)
2008
15
Paraneoplastic pemphigus associated with non-Hodgkin lymphoma. (18713592)
2008
16
Paraneoplastic pemphigus. (17683379)
2007
17
Paraneoplastic pemphigus associated with Castleman's disease: usefulness of the laboratory of autoimmunity in the diagnosis of this disease. (17804551)
2007
18
A novel case of IgA paraneoplastic pemphigus associated with chronic lymphocytic leukemia. (17434044)
2007
19
Retroperitoneal Castleman's tumor and paraneoplastic pemphigus: report of a case and review of the literature. (17466075)
2007
20
Paraneoplastic pemphigus in association with B-cell lymphocytic leukemia and hepatitis C: favorable response to intravenous immunoglobulins and prednisolone. (17614814)
2007
21
Severe gastrointestinal involvement in paraneoplastic pemphigus. (16935802)
2006
22
Paraneoplastic pemphigus revealing dendritic cell sarcoma originating from Castleman's disease of the neck]. (15746607)
2005
23
Paraneoplastic pemphigus: a case report and review of literature. (16120122)
2005
24
Neoplasms associated with paraneoplastic pemphigus: a review with emphasis on non-hematologic malignancy and oral mucosal manifestations. (15063382)
2004
25
Paraneoplastic pemphigus. (14870982)
2004
26
Using rituximab (anti-CD20 antibody) in a patient with paraneoplastic pemphigus. (14558407)
2003
27
Paraneoplastic pemphigus is associated with the DRB1*03 allele. (12604316)
2003
28
Paraneoplastic pemphigus associated with Castleman's disease and asymptomatic bronchiolitis obliterans. (12095881)
2002
29
Paraneoplastic pemphigus sera react strongly with multiple epitopes on the various regions of envoplakin and periplakin, except for the c-terminal homologous domain of periplakin. (11286623)
2001
30
Immunopathology of paraneoplastic pemphigus. (11705675)
2001
31
Classification, clinical manifestations, and immunopathological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome: a reappraisal of paraneoplastic pemphigus. (11176692)
2001
32
A case of paraneoplastic pemphigus with antidesmoglein 1 antibodies as determined by immunoblotting. (10792239)
2000
33
Periplakin and envoplakin are target antigens in canine and human paraneoplastic pemphigus. (10321593)
1999
34
Human autoantibodies against HD1/plectin in paraneoplastic pemphigus. (9989789)
1999
35
The mechanism of respiratory failure in paraneoplastic pemphigus. (10490388)
1999
36
Novel non-radioisotope immunoprecipitation studies indicate involvement of pemphigus vulgaris antigen in paraneoplastic pemphigus. (9673895)
1998
37
Autoantibodies from a patient with paraneoplastic pemphigus bind periplakin, a novel member of the plakin family. (9699741)
1998
38
Paraneoplastic pemphigus associated with Hodgkin's disease. (9536253)
1998
39
Bronchiolitis obliterans, Castleman's disease, and a bullous disease: pemphigus vulgaris or paraneoplastic pemphigus? (9635692)
1998
40
The members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin. (9699735)
1998
41
Paraneoplastic pemphigus treated with dexamethasone/ cyclophosphamide pulse therapy. (9889426)
1998
42
cDNA cloning of the 210-kDa paraneoplastic pemphigus antigen reveals that envoplakin is a component of the antigen complex. (9284106)
1997
43
Paraneoplastic pemphigus associated with pancreatic carcinoma. (9228221)
1997
44
Paraneoplastic pemphigus: potential therapeutic effect of plasmapheresis. (8736359)
1996
45
Accuracy of indirect immunofluorescence testing in the diagnosis of paraneoplastic pemphigus. (7868713)
1995
46
Treatment with alpha interferon associated with the development of paraneoplastic pemphigus. (7718471)
1995
47
Paraneoplastic pemphigus presenting as a lichen planus pemphigoides-like eruption. (8323308)
1993
48
Immunofluorescence and immunoelectron microscopy analyses of a human monoclonal anti-epithelial cell surface antibody that recognizes a 185-kD polypeptide: a component of the paraneoplastic pemphigus antigen complex? (8370971)
1993
49
Paraneoplastic pemphigus. A distinct autoimmune vesiculobullous disorder associated with neoplasia. (8426720)
1993
50
Paraneoplastic pemphigus, cicatricial conjunctivitis, and acanthosis nigricans with pachydermatoglyphy in a patient with bronchogenic squamous cell carcinoma. (1741121)
1992

Genetic Variations for Paraneoplastic Pemphigus

Expression for genes affiliated with Paraneoplastic Pemphigus

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
4Cell Signaling Technology, 54Reactome, 30KEGG, 38NCBI BioSystems Database, 12EMD Millipore, 52QIAGEN
See all sources

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
Hide members
9.6CD79A, HLA-DRB1, HLA-DQB1
69.5PLEC, DST, DSP, COL17A1
7
Hide members
9.5COL17A1, JUP, DST, PLEC
8
Immune response IFN alpha/beta signaling pathway
Hide members
9.3HLA-A, HLA-B, HLA-C
9
Hide members
9.3HLA-A, HLA-B, HLA-C
10
Hide members
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
Hide members
9.0HLA-C, HLA-B, HLA-A, CD79A
16
Hide members
9.0DSP, DSG3, DSG1, DSG2, PLEC
17
Hide members
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
Hide members
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
Hide members
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

Sources:
45Novoseek, 11DrugBank, 24HMDB, 50PharmGKB, 29IUPHAR
See all sources

Compounds related to Paraneoplastic Pemphigus according to GeneCards/GeneDecks:

(show all 14)
idCompoundScoreTop Affiliating Genes
1dr-104510.0HLA-A, HLA-DRB1
2asulam4510.0DSP, PPL
3pneumococcal459.8HLA-B, CD79A
4toxin a459.8DSG3, DSG1
5ivig459.5DSG3, DSG1, CD79A
6allergens459.5CD79A, BLZF1, HLA-DRB1
7sucrose45 11 2411.3COL17A1, JUP, DSP, DSG1, BLZF1
8oligonucleotide458.8JUP, HLA-DQB1, HLA-B, HLA-DRB1, HLA-A, DST
9steroid458.8CD79A, BLZF1, DSP, HLA-B, COL17A1
10acetylcholine45 50 29 11 2412.7CD79A, BLZF1, DSG1, DSG3
11retinoic acid45 249.7CD79A, BLZF1, DSG1, DSP, HLA-B, JUP
12serine458.3COL17A1, JUP, HLA-B, DSP, DSG3, DSG1
13calcium45 50 11 2410.2DSP, C6orf25, JUP, COL17A1, DSG3, DST
14tyrosine456.5BLZF1, COL17A1, JUP, HLA-DQB1, HLA-B, HLA-DRB1

GO Terms for genes affiliated with Paraneoplastic Pemphigus

Sources:
16Gene Ontology
See all sources

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

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Paraneoplastic Pemphigus

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet