MCID: PRN049
MIFTS: 41

Paraneoplastic Pemphigus malady

Categories: Skin diseases, Rare diseases

Aliases & Classifications for Paraneoplastic Pemphigus

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Sources:
51Orphanet, 65UMLS, 28ICD10 via Orphanet, 66UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Paraneoplastic Pemphigus:

Name: Paraneoplastic Pemphigus 51 65

Characteristics:

Orphanet epidemiological data:

51
paraneoplastic pemphigus:
Inheritance: Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: All ages; Age of death: any age

Classifications:

Orphanet: 51 
Rare skin diseases


External Ids:

Orphanet51 63455
ICD10 via Orphanet28 L10.8
UMLS via Orphanet66 C1112570
UMLS65 C1112570

Summaries for Paraneoplastic Pemphigus

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Wikipedia:68 Paraneoplastic pemphigus is an autoimmune disorder stemming from an underlying tumor. It is hypothesized... more...

MalaCards based summary: Paraneoplastic Pemphigus is related to lichen planus pemphigoides and pemphigus vulgaris, and has symptoms including abnormality of the oral cavity, abnormal blistering of the skin and vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment. An important gene associated with Paraneoplastic Pemphigus is EVPL (Envoplakin), and among its related pathways are Cytoskeleton remodeling Neurofilaments and Staphylococcus aureus infection. Affiliated tissues include skin, heart and breast.

Related Diseases for Paraneoplastic Pemphigus

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

(show top 50)    (show all 159)
idRelated DiseaseScoreTop Affiliating Genes
1lichen planus pemphigoides30.5HLA-DQB1, HLA-DRB1
2pemphigus vulgaris30.1DSG1, DSG2, DSG3, EVPL, HLA-DRB1, PPL
3human herpesvirus 829.7HLA-A, HLA-B, HLA-C
4pemphigus11.3
5pili gemini10.8DSG3, DSP
6braddock syndrome10.7DSG1, DSP
7intellectual disability-sparse hair-brachydactyly syndrome10.7DSG1, DSG3
8acute opioid poisoning10.7DSG1, DSG3
9hypercholesterolemia, familial10.7DSC2, DSP
10pancreatic intraductal papillary-mucinous adenoma10.6DSG1, DSP
11arrhythmogenic right ventricular dysplasia 1210.6DSC2, DSP
12microsomia hemifacial radial defects10.5HLA-B, HLA-DRB1
13hemorrhagic fever with renal syndrome10.5HLA-C, HLA-DRB1
14hypotrichosis and recurrent skin vesicles10.5DSC2, DSC3
15lymphoma10.5
16drug-induced mental disorder10.5HLA-C, HLA-DRB1
17familial isolated arrhythmogenic ventricular dysplasia, left dominant form10.4DSC2, DSG2, DSP
18frontotemporal dementia, right temporal atrophy variant10.4DSC2, DSG2, DSP
19mitf-related melanoma and renal cell carcinoma predisposition syndrome10.4DSC2, DSG2, DSP
20microcystic lymphatic malformation10.4DSC3, DSG1, PPL
21bronchiolitis10.4
22bronchiolitis obliterans10.4
23specific developmental disorder10.4DSC2, DSG2, DSP
24tucker syndrome10.4HLA-DQB1, HLA-DRB1
25periventricular leukomalacia10.4HLA-A, HLA-B
26heavy chain deposition disease10.3HLA-DQB1, HLA-DRB1
27sarcoma10.3
28pulmonary coin lesion10.3HLA-DQB1, HLA-DRB1
29asperger syndrome10.3DSC2, DSG2, DSP
30hydromyelia10.3HLA-A, HLA-B
31autoimmune polyglandular syndrome type 210.3HLA-DQB1, HLA-DRB1
32best1 retinopathy10.3HLA-DQB1, HLA-DRB1
33retinitis10.3HLA-A, HLA-B
34non-distal monosomy 10q10.3HLA-DQB1, HLA-DRB1
35dendritic cell sarcoma10.3
36lichtenstein syndrome10.3HLA-DQB1, HLA-DRB1
37anthracosis10.3HLA-A, HLA-B
38red cell phospholipid defect with hemolysis10.3HLA-DQB1, HLA-DRB1
39undetermined early-onset epileptic encephalopathy10.3HLA-A, HLA-B
40follicular dendritic cell sarcoma10.3
41prostate malignant phyllodes tumor10.3HLA-A, HLA-B
42actinomycosis10.3HLA-DQB1, HLA-DRB1
43hypercementosis10.3HLA-B, HLA-DRB1
44acquired thrombocytopenia10.2HLA-A, HLA-B
45pervasive developmental disorder10.2DSG1, DSP
46leukemia10.2
47thymoma10.2
48lichen planus10.2
49castleman disease10.2
50finnish upper limb-onset distal myopathy10.2HLA-DQB1, HLA-DRB1

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



Diseases related to paraneoplastic pemphigus

Symptoms for Paraneoplastic Pemphigus

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Symptoms:

 51
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • enanthema/aphtosa/aphta/leukoplakia

HPO human phenotypes related to Paraneoplastic Pemphigus:

id Description Frequency HPO Source Accession
1 abnormality of the oral cavity hallmark (90%) HP:0000163
2 abnormal blistering of the skin hallmark (90%) HP:0008066

UMLS symptoms related to Paraneoplastic Pemphigus:


coma blister

Drugs & Therapeutics for Paraneoplastic Pemphigus

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Interventional clinical trials:

Search ClinicalTrials, NIH Clinical Center for Paraneoplastic Pemphigus

Genetic Tests for Paraneoplastic Pemphigus

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Anatomical Context for Paraneoplastic Pemphigus

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MalaCards organs/tissues related to Paraneoplastic Pemphigus:

33
Skin, Heart, Breast, T cells, B cells, Prostate, Lung

Animal Models for Paraneoplastic Pemphigus or affiliated genes

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MGI Mouse Phenotypes related to Paraneoplastic Pemphigus:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes

Publications for Paraneoplastic Pemphigus

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Articles related to Paraneoplastic Pemphigus:

(show top 50)    (show all 316)
idTitleAuthorsYear
1
Clinical and laboratory characterization of 114 cases of Castleman disease patients from a single centre: paraneoplastic pemphigus is an unfavourable prognostic factor. (25824806)
2015
2
Gastric Cancer and Paraneoplastic Pemphigus. (26884668)
2015
3
Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma: report of a case and review of literature. (26722384)
2015
4
Paraneoplastic pemphigus associated with primary pulmonar follicular dendritic cell sarcoma showing good response to treatment. (25388966)
2014
5
The importance of multidisciplinary healthcare for paraneoplastic pemphigus. (25257550)
2014
6
Detection of autoantibodies to periplakin and envoplakin in paraneoplastic pemphigus but not idiopathic pulmonary fibrosis using full-length recombinant proteins. (24275244)
2013
7
IgA pemphigus with paraneoplastic pemphigus-like clinical features showing IgA antibodies to desmoglein 1/3 and desmocollin 3, and IgG and IgA antibodies to the basement membrane zone. (23517469)
2013
8
Laboratory diagnosis of paraneoplastic pemphigus. (23796242)
2013
9
Interdigitating dendritic cell sarcoma complicated with paraneoplastic pemphigus after surgery of resection. (23652075)
2013
10
Paraneoplastic pemphigus: initial manifestation of lung cancer. (23670336)
2013
11
A case report of paraneoplastic pemphigus associated with esophageal squamous cell carcinoma. (23613673)
2013
12
Bronchiolitis obliterans presenting as prolonged ventilator dependence in a patient with paraneoplastic pemphigus. (22813514)
2012
13
Paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome): clinical presentations and pathogenesis. (21605807)
2011
14
Improved outcomes after complete resection of underlying tumors for patients with paraneoplastic pemphigus: a single-center experience of 22 cases. (20390428)
2011
15
Paraneoplastic pemphigus with Castleman's disease and bronchiolitis obliterans. (22181577)
2011
16
Paraneoplastic pemphigus associated with corneal perforation and cutaneous alternariosis: a case report and review of cases treated with rituximab. (21434987)
2011
17
Paraneoplastic pemphigus associated with malignant gastrointestinal stromal tumour. (20107737)
2010
18
The protease inhibitor alpha-2-macroglobulin-like-1 is the p170 antigen recognized by paraneoplastic pemphigus autoantibodies in human. (20805888)
2010
19
Fatal paraneoplastic pemphigus associated with a mediastinal tumor. (19087213)
2009
20
A case of paraneoplastic pemphigus associated with Castleman's disease presenting as erosive lichen planus. (19663833)
2009
21
Detection and characterization of anti-envoplakin linker autoantibodies in paraneoplastic pemphigus using specific bead-based assay. (18790674)
2008
22
Paraneoplastic pemphigus. (17683379)
2007
23
Paraneoplastic pemphigus without an underlying neoplasm. (17300250)
2007
24
Paraneoplastic pemphigus associated with Castleman's disease: usefulness of the laboratory of autoimmunity in the diagnosis of this disease. (17804551)
2007
25
Paraneoplastic Pemphigus and Bronchiolitis Obliterans in a Patient with Splenic B-cell Lymphoma. (17908666)
2007
26
Paraneoplastic pemphigus in a dog with splenic sarcoma. (15657279)
2005
27
Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma and Castleman disease. (16029358)
2005
28
Exacerbation of paraneoplastic pemphigus by cyclophosphamide treatment: detection of novel autoantigens and bronchial autoantibodies. (15149520)
2004
29
Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) in a child: case report and review of the literature. (15466078)
2004
30
No involvement of IgG autoantibodies against extracellular domains of desmoglein 2 in paraneoplastic pemphigus or inflammatory bowel diseases. (12850306)
2003
31
Herpetiformis pemphigus and lung carcinoma: a case of paraneoplastic pemphigus. (12361141)
2002
32
Paraneoplastic pemphigus triggered by Castleman's disease. (11903261)
2002
33
Paraneoplastic pemphigus: a refractory autoimmune mucocutaneous disease. (12196890)
2002
34
Paraneoplastic pemphigus. (11589740)
2001
35
Paraneoplastic pemphigus. A report of two cases associated with chronic B-cell lymphocytic leukaemia. (11453921)
2001
36
Paraneoplastic pemphigus with circulating antibodies directed exclusively against the pemphigus vulgaris antigen desmoglein 3. (11004638)
2000
37
Paraneoplastic pemphigus in two patients with B-cell non-Hodgkin's lymphoma: significant responses to cyclophosphamide and prednisolone. (10629583)
2000
38
Periplakin and envoplakin are target antigens in canine and human paraneoplastic pemphigus. (10321593)
1999
39
Plectin serves as an autoantigen in paraneoplastic pemphigus. (10469345)
1999
40
Graft-versus-host disease-like immunophenotype and apoptotic keratinocyte death in paraneoplastic pemphigus. (10583130)
1999
41
Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. (9710446)
1998
42
Novel non-radioisotope immunoprecipitation studies indicate involvement of pemphigus vulgaris antigen in paraneoplastic pemphigus. (9673895)
1998
43
Childhood paraneoplastic pemphigus associated with Castleman's tumour. (9039308)
1997
44
cDNA cloning of the 210-kDa paraneoplastic pemphigus antigen reveals that envoplakin is a component of the antigen complex. (9284106)
1997
45
Paraneoplastic pemphigus: report of a case. (8708152)
1996
46
Lethal paraneoplastic pemphigus following treatment of chronic lymphocytic leukaemia with fludarabine. (8664199)
1995
47
Paraneoplastic pemphigus presenting as a lichen planus pemphigoides-like eruption. (8323308)
1993
48
Indirect immunofluorescence on rat bladder transitional epithelium: a test with high specificity for paraneoplastic pemphigus. (7684408)
1993
49
Human autoantibodies against desmoplakins in paraneoplastic pemphigus. (1601988)
1992
50

Variations for Paraneoplastic Pemphigus

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Expression for genes affiliated with Paraneoplastic Pemphigus

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Search GEO for disease gene expression data for Paraneoplastic Pemphigus.

Pathways for genes affiliated with Paraneoplastic Pemphigus

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Pathways related to Paraneoplastic Pemphigus according to GeneCards Suite gene sharing:

(show top 14)    (show all 15)
idSuper pathwaysScoreTop Affiliating Genes
1
Show member pathways
9.8DSP, EVPL, PPL
29.4DSG1, HLA-DQB1, HLA-DRB1
39.3HLA-A, HLA-B, HLA-C
4
Show member pathways
9.3HLA-A, HLA-B, HLA-C
59.3HLA-A, HLA-B, HLA-C
6
Show member pathways
9.2DSC2, DSC3, DSG2, DSP
7
Show member pathways
8.5HLA-A, HLA-B, HLA-C, HLA-DQB1, HLA-DRB1
88.5HLA-A, HLA-B, HLA-C, HLA-DQB1, HLA-DRB1
9
Show member pathways
8.5HLA-A, HLA-B, HLA-C, HLA-DQB1, HLA-DRB1
108.5HLA-A, HLA-B, HLA-C, HLA-DQB1, HLA-DRB1
11
Show member pathways
8.5HLA-A, HLA-B, HLA-C, HLA-DQB1, HLA-DRB1
128.5HLA-A, HLA-B, HLA-C, HLA-DQB1, HLA-DRB1
138.5HLA-A, HLA-B, HLA-C, HLA-DQB1, HLA-DRB1
14
Show member pathways
8.5HLA-A, HLA-B, HLA-C, HLA-DQB1, HLA-DRB1

GO Terms for genes affiliated with Paraneoplastic Pemphigus

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Cellular components related to Paraneoplastic Pemphigus according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1integral component of lumenal side of endoplasmic reticulum membraneGO:007155610.2HLA-C, HLA-DQB1
2intercalated discGO:001470410.2DSC2, DSG2
3ER to Golgi transport vesicle membraneGO:00125079.3HLA-A, HLA-C, HLA-DQB1, HLA-DRB1
4cell surfaceGO:00099869.3DSG2, HLA-A, HLA-B, HLA-C
5plasma membraneGO:00058866.7C6orf25, DSC1, DSC2, DSC3, DSG1, DSG2

Biological processes related to Paraneoplastic Pemphigus according to GeneCards Suite gene sharing:

(show all 10)
idNameGO IDScoreTop Affiliating Genes
1bundle of His cell-Purkinje myocyte adhesion involved in cell communicationGO:008607310.3DSC2, DSG2
2immunoglobulin production involved in immunoglobulin mediated immune responseGO:000238110.3HLA-DQB1, HLA-DRB1
3protection from natural killer cell mediated cytotoxicityGO:004227010.2HLA-A, HLA-B
4positive regulation of T cell mediated cytotoxicityGO:000191610.2HLA-A, HLA-B
5regulation of heart rate by cardiac conductionGO:008609110.2DSG2, DSP
6antigen processing and presentation of peptide antigen via MHC class IGO:00024749.6HLA-A, HLA-B, HLA-C
7interferon-gamma-mediated signaling pathwayGO:00603339.6HLA-A, HLA-B, HLA-C
8homophilic cell adhesion via plasma membrane adhesion moleculesGO:00071569.2DSC2, DSG2, DSG3
9antigen processing and presentationGO:00198828.9HLA-A, HLA-B, HLA-C, HLA-DQB1, HLA-DRB1
10apoptotic processGO:00069158.7DSG1, DSG2, DSG3, DSP

Molecular functions related to Paraneoplastic Pemphigus according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1peptide antigen bindingGO:00426059.6HLA-A, HLA-B

Sources for Paraneoplastic Pemphigus

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet