MCID: PMP001
MIFTS: 60

Pemphigus malady

Rare diseases, Skin diseases categories
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Summaries for Pemphigus

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43NIH Rare Diseases, 34MedlinePlus, 65Wikipedia, 33MalaCards
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MedlinePlus:34 Pemphigus is an autoimmune disorder. if you have it, your immune system attacks healthy cells in your skin and mouth, causing blisters and sores. no one knows the cause. pemphigus does not spread from person to person. it does not appear to be inherited. but some people's genes put them more at risk for pemphigus. pemphigoid is also an autoimmune skin disease. it leads to deep blisters that do not break easily. pemphigoid is most common in older adults and may be fatal for older, sick patients. doctors diagnose pemphigus with a physical exam, a biopsy, and blood tests. the treatment of pemphigus and pemphigoid is the same: one or more medicines to control symptoms. these may include steroids, which reduce inflammation drugs that suppress the immune system response antibiotics to treat associated infections nih: national institute of arthritis and musculoskeletal and skin diseases

MalaCards: Pemphigus, also known as pemphigus nos, is related to pemphigus foliaceus and pemphigus vulgaris. An important gene associated with Pemphigus is DSG1 (desmoglein 1), and among its related pathways are Staphylococcus aureus infection and Apoptotic cleavage of cellular proteins. The drugs auranofin and chloroquine and the compounds ivig and 12-o-tetradecanoylphorbol 13-acetate have been mentioned in the context of this disorder. Affiliated tissues include skin, testes and t cells, and related mouse phenotypes are growth/size/body and cellular.

NIH Rare Diseases:43 Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and mucous membranes (mouth, nose, throat, eyes, and genitals). this condition can occur at any age, but often strikes people in middle or older age. it is more common in people of middle eastern or jewish descent than in other races and cultures. pemphigus is a chronic disease which is best controlled by early diagnosis and treatment. treatment includes steroids to reduce inflammation, drugs that suppress the immune system response and antibiotics to treat associated infections. there are three main types of pemphigus: pemphigus vulgaris pemphigus foliaceus  paraneoplastic pemphigus last updated: 3/3/2010

Wikipedia:65 Pemphigus (/?p?mf???s/ or /p?m?fa???s/) is a rare group of blistering autoimmune diseases that affect... more...

Aliases & Classifications for Pemphigus

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8Disease Ontology, 43NIH Rare Diseases, 45Novoseek, 34MedlinePlus, 62UMLS, 35MeSH, 27ICD9CM, 58SNOMED-CT, 40NCIt, 25ICD10
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Classifications:

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


Aliases & Descriptions:

pemphigus 8 43 45 34 62
pemphigus nos 8


External Ids:

Disease Ontology8 DOID:9182
MeSH35 D010392
ICD9CM27 694.4
NCIt40 C34909
ICD1025 L10, L10.9

Related Diseases for Pemphigus

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

(show top 50)    (show all 242)
idRelated DiseaseScoreTop Affiliating Genes
1pemphigus foliaceus31.6DST, HLA-DRB1, DSG1, DSG2, PLAUR, EVPL
2pemphigus vulgaris31.5DST, DSG1, DSP, JUP, HLA-DRB1, DSG2
3bullous pemphigoid31.1DST, EVPL, DSP
4hailey-hailey disease31.0DSP, JUP, ATP2C1
5thymoma30.8EVPL, DSP
6sarcoma30.6DSG1, EVPL, DSP, JUP
7impetigo30.6DSG4
8subcorneal pustular dermatosis30.6DSG1, CD79A, DSC3
9cicatricial pemphigoid30.5DST, CD79A
10skin disease30.4HLA-DRB1
11thyroiditis30.4HLA-DRB1, CD79A
12leukemia30.4EVPL, HLA-DRB1, CD79A, PLAUR
13herpes gestationis30.1DST
14connective tissue disease30.0CD79A, HLA-DRB1
15keratosis30.0DSP, JUP
16epidermolysis bullosa29.8DST, DSP
17melanoma29.8HLA-DRB1, JUP, DST, DSG1, PLAUR
18ulcerative colitis29.7HLA-DRB1, CD79A, JUP
19paraneoplastic pemphigus11.1
20pemphigus vegetans10.9
21pemphigus erythematosus10.8
22herpetiform pemphigus10.5
23castleman's disease10.5
24lichen planus10.4
25lupus erythematosus10.4
26bronchiolitis10.4
27psoriasis10.4
28bronchiolitis obliterans10.4
29dermatitis10.4
30myasthenia gravis10.4
31mucous membrane pemphigoid10.4
32pemphigus and fogo selvagem10.4
33dendritic cell sarcoma10.3
34hodgkin's lymphoma10.3
35superficial pemphigus10.3
36follicular dendritic cell sarcoma10.3
37usher syndrome10.3
38systemic lupus erythematosus10.3
39chronic lymphocytic leukemia10.3
40esophagitis10.2
41b-cell lymphomas10.2
42paronychia10.2
43gingivitis10.2
44bullous impetigo10.2
45kaposi's sarcoma10.2
46acanthosis nigricans10.2
47alopecia10.2
48herpes simplex10.2
49respiratory failure10.2
50stomatitis10.2

Graphical network of the top 20 diseases related to Pemphigus:



Diseases related to pemphigus

Symptoms for Pemphigus

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Drugs & Therapeutics for Pemphigus

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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Pemphigus

Search NIH Clinical Center for Pemphigus

Inferred drug relations via UMLS62/NDF-RT41:

Genetic Tests for Pemphigus

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Anatomical Context for Pemphigus

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33MalaCards
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MalaCards organs/tissues related to Pemphigus:

33
Skin, Testes, T cells, Eye, B cells, Lung, Tongue, Thyroid, Bone, Colon, Bone marrow, Thymus, Prostate, Lymph node, Myeloid, Kidney, Bronchial epithelium

Animal Models for Pemphigus or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Pemphigus:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053787.6ATP2C1, JUP, DSP, DST, DSG4, PLAUR
2MP:00053847.4CD79A, ATP2C1, JUP, DSP, DSG2, PLAUR
3MP:00107717.3ATP2C1, JUP, DSP, DST, DSG4, DSC3
4MP:00107687.1DSC3, ATP2C1, JUP, DSP, DST, DSG4

Publications for Pemphigus

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52PubMed
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Articles related to Pemphigus:

(show top 50)    (show all 1933)
idTitleAuthorsYear
1
Biomarkers of oxidative stress in epidermis of Tunisian pemphigus foliaceus patients. (22738420)
2013
2
Both qualitative and quantitative genetic variation of MHC class II molecules may influence susceptibility to autoimmune diseases: the case of endemic pemphigus foliaceus. (23777927)
2013
3
Detection of autoantibodies to periplakin and envoplakin in paraneoplastic pemphigus but not idiopathic pulmonary fibrosis using full-length recombinant proteins. (24275244)
2013
4
Prognostic factors in pemphigus vulgaris and pemphigus foliaceus. (24102444)
2013
5
Laboratory diagnosis of herpesvirus infections in patients with Pemphigus vulgaris lesions. (23735576)
2013
6
Signaling dependent and independent mechanisms in pemphigus vulgaris blister formation. (23226536)
2012
7
Acute zoster in known pemphigus vulgaris and bullous pemphigoid: avoiding the "disease flare" trap. (21571161)
2011
8
Paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome): clinical presentations and pathogenesis. (21605807)
2011
9
Prevalence and treatment of pemphigus in Iran. (21925018)
2011
10
Pemphigus vulgaris antigen mRNA quantification for the staging of sentinel lymph nodes in head and neck cancer. (19997107)
2010
11
The efficacy of nicotinamide gel 4% as an adjuvant therapy in the treatment of cutaneous erosions of pemphigus vulgaris. (20597951)
2010
12
Familial benign chronic pemphigus (Hailey-Hailey disease). (21163165)
2010
13
ABO blood groups and pemphigus vulgaris: no relationship. (20372776)
2010
14
Palm tissue displaying a polyclonal autoimmune response in patients affected by a new variant of endemic pemphigus foliaceus in Colombia, South America. (19889592)
2010
15
Binding of pemphigus vulgaris IgG to antigens in desmosome core domains excludes immune complexes rather than directly splitting desmosomes. (20222919)
2010
16
An imbalance in Akt/mTOR is involved in the apoptotic and acantholytic processes in a mouse model of pemphigus vulgaris. (19552768)
2009
17
Autoantibodies against desmocollins in European patients with pemphigus. (19456767)
2009
18
Loss of the desmosomal protein perp enhances the phenotypic effects of pemphigus vulgaris autoantibodies. (19158843)
2009
19
Antigen selection of anti-DSG1 autoantibodies during and before the onset of endemic pemphigus foliaceus. (19571823)
2009
20
Canine pemphigus foliaceus antigen is localized within desmosomes of keratinocyte. (18976819)
2009
21
Paraneoplastic pemphigus. (17683379)
2007
22
Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment. (17461818)
2007
23
Detection of pemphigus desmoglein 1 and desmoglein 3 autoantibodies and pemphigoid BP180 autoantibodies in saliva and comparison with serum values. (17026501)
2006
24
Direct immunofluorescence on uninvolved, lesional and perilesional skin in patients with endemic pemphigus foliaceus (fogo selvagem). (15567982)
2004
25
Mucosal dominant pemphigus vulgaris with anti-desmoplakin autoantibodies. (15243525)
2004
26
Exacerbation of paraneoplastic pemphigus by cyclophosphamide treatment: detection of novel autoantigens and bronchial autoantibodies. (15149520)
2004
27
Topical 0.1% tacrolimus for the treatment of discoid lupus erythematosus and pemphigus erythematosus in dogs. (14736903)
2004
28
Association between HLA-DRB1, DQB1 genes and pemphigus vulgaris in Chinese Hans]. (12579512)
2003
29
Commentary/millenium: endemic pemphigus foliaceus (fogo selvagem). (11123439)
2000
30
Paraneoplastic pemphigus with circulating antibodies directed exclusively against the pemphigus vulgaris antigen desmoglein 3. (11004638)
2000
31
Analysis of the T cells that are potentially involved in autoantibody production in pemphigus vulgaris. (10635617)
1999
32
Antibodies to desmoplakin in a patient with pemphigus foliaceous. (9784047)
1998
33
Diet and pemphigus. In pursuit of exogenous factors in pemphigus and fogo selvagem. (9828876)
1998
34
Pemphigus IgG induces expression of urokinase plasminogen activator receptor on the cell surface of cultured keratinocytes. (9347794)
1997
35
Childhood paraneoplastic pemphigus associated with Castleman's tumour. (9039308)
1997
36
Mucosal and mucocutaneous (generalized) pemphigus vulgaris show distinct autoantibody profiles. (9326396)
1997
37
Herpetiform pemphigus showing reactivity with pemphigus vulgaris antigen (desmoglein 3) (9274636)
1997
38
Cloning of the gene for human pemphigus vulgaris antigen (desmoglein 3), a desmosomal cadherin. Characterization of the promoter region and identification of a keratinocyte-specific cis-element. (8663392)
1996
39
Pemphigus foliaceus and pemphigus vulgaris autoantibodies react with the extracellular domain of desmoglein-1. (7860995)
1995
40
Atypical pemphigus with concomitant IgG and IgA anti-intercellular autoantibodies associated with monoclonal IgA gammopathy. (8049548)
1994
41
Pemphigus foliaceus treated successfully with plasma exchange. (1478768)
1992
42
Ultrastructural binding site of pemphigus foliaceus autoantibodies: comparison with pemphigus vulgaris. (1918503)
1991
43
Pemphigus vegetans presenting with deafness, otalgia and facial nerve paralysis. (2064948)
1991
44
Pemphigus foliaceus with anti-intercellular and anti-basement membrane zone antibodies. Blocking immunofluorescence and Western immunoblotting studies. (3069506)
1988
45
Sequence analysis of the HLA-DR beta and HLA-DQ beta loci from three Pemphigus vulgaris patients. (3391803)
1988
46
A newly characterized HLA DQ beta allele associated with pemphigus vulgaris. (2894075)
1988
47
Pemphigus foliaceus coexisting with toxic, multinodular goiter. (3771047)
1986
48
Pemphigus vegetans. Neumann type and Hallopeau type. (6365813)
1984
49
Brazilian pemphigus foliaceus. (7085163)
1982
50
PEMPHIGUS ERYTHEMATOSUS; FAVORABLE RESPONSE TO TOPICAL TREATMENT. (14170605)
1964

Variations for Pemphigus

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Expression for genes affiliated with Pemphigus

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pemphigus

Search GEO for disease gene expression data for Pemphigus.

Pathways for genes affiliated with Pemphigus

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50PathCards, 30KEGG, 55Reactome, 38NCBI BioSystems Database, 60Thomson Reuters, 51PharmGKB, 5Cell Signaling Technology
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Compounds for genes affiliated with Pemphigus

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45Novoseek, 24HMDB, 11DrugBank, 51PharmGKB
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Compounds related to Pemphigus according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1ivig459.8CD79A, DSG1
212-o-tetradecanoylphorbol 13-acetate459.2PLAUR, DST, DSP
3sucrose45 24 1110.8PLAUR, DSG1, DSP, JUP
4paraffin458.5JUP, PLAUR, CD79A, DST
5oligonucleotide458.3DST, HLA-DRB1, JUP, PLAUR
6retinoic acid45 249.2JUP, DSP, DSG1, CD79A, PLAUR
7estrogen458.1JUP, CD79A, PLAUR, ATP2C1
8tyrosine457.8ATP2C1, PLAUR, HLA-DRB1, DSG2, DSP, JUP
9calcium45 51 24 119.6PLAUR, ATP2C1, JUP, DSP, DST, DSG4

GO Terms for genes affiliated with Pemphigus

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

(show all 12)
idNameGO IDScoreTop Affiliating Genes
1cornified envelopeGO:0015339.9DSP, EVPL
2fascia adherensGO:0059169.4JUP, DSP
3hemidesmosomeGO:0300569.4JUP, DST
4cytoplasmic side of plasma membraneGO:0098989.4JUP, DSG1
5lateral plasma membraneGO:0163289.2DSG2, DSG1, JUP
6intermediate filamentGO:0058829.2DSP, DST, JUP
7intercalated discGO:0147049.1JUP, DSP
8cell-cell junctionGO:0059118.7JUP, DSG1, DSG2, DSC3
9extracellular vesicular exosomeGO:0700628.1JUP, DSP, DSG2, EVPL, HLA-DRB1
10desmosomeGO:0300577.5DSC3, EVPL, DSP, DSG2, DSG1, DSG4
11integral component of membraneGO:0160217.3ATP2C1, DSG4, DSG1, DSG2, DSC3, PLAUR
12plasma membraneGO:0058866.4HLA-DRB1, JUP, DSP, DSG4, DSG1, DSG2

Biological processes related to Pemphigus according to GeneCards/GeneDecks:

(show all 13)
idNameGO IDScoreTop Affiliating Genes
1maternal process involved in female pregnancyGO:0601359.9DSG1, DSG2
2peptide cross-linkingGO:0181499.9DSP, EVPL
3calcium-dependent cell-cell adhesionGO:0163399.8DSG1, ATP2C1
4cellular component disassembly involved in execution phase of apoptosisGO:0069219.8DSG2, DSG1, DSP
5epidermis developmentGO:0085449.7EVPL, DSP, ATP2C1
6response to progesteroneGO:0325709.5DSG1, DSG2
7keratinocyte differentiationGO:0302169.4EVPL, DSP, DSG4
8adherens junction organizationGO:0343329.4JUP, DSP
9bundle of His cell to Purkinje myocyte communicationGO:0860699.3JUP, DSG2, DSP
10ventricular cardiac muscle cell action potentialGO:0860059.3DSG2, DSP, JUP
11regulation of heart rate by cardiac conductionGO:0860919.2JUP, DSG2, DSP
12cell-cell adhesionGO:0163379.2DSG1, DSP, JUP
13homophilic cell adhesionGO:0071568.9DSG4, DSG1, DSG2, DSC3

Molecular functions related to Pemphigus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein binding, bridgingGO:0306749.9EVPL, DSP
2gamma-catenin bindingGO:0452959.6DSC3, DSG1
3structural molecule activityGO:0051988.8JUP, DSP, EVPL
4calcium ion bindingGO:0055098.0DSG4, ATP2C1, DSC3, DSG2, DSG1, DST

Products for genes affiliated with Pemphigus

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Sources for Pemphigus

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4CDC
13ExPASy
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22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
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40NCIt
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44NINDS
45Novoseek
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48OMIM via Orphanet
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59SNOMED-CT via Orphanet
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63UMLS via Orphanet