MCID: PMP001
MIFTS: 60

Pemphigus malady

Rare diseases, Skin diseases categories
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Summaries for Pemphigus

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MedlinePlus:33 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 based summary: Pemphigus is related to bullous pemphigoid and pemphigus foliaceus. 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 chloroquine and chloroquine phosphate 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:42 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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Pemphigus, Aliases & Descriptions:

Name: Pemphigus 8 42 44 33 62


Classifications:

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


External Ids:

Disease Ontology8 DOID:9182
NCIt39 C34909
MeSH34 D010392
ICD9CM27 694.4
ICD1025 L10, L10.9

Related Diseases for Pemphigus

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

(show top 50)    (show all 247)
idRelated DiseaseScoreTop Affiliating Genes
1bullous pemphigoid31.4DST, EVPL, DSP
2pemphigus foliaceus31.1DST, HLA-DRB1, DSG1, DSG2, PLAUR, EVPL
3hailey-hailey disease31.1DSP, JUP, ATP2C1
4thymoma31.0EVPL, DSP
5impetigo31.0DSG4
6mucous membrane pemphigoid31.0DST, CD79A
7skin disease30.8HLA-DRB1
8subcorneal pustular dermatosis30.8DSG1, CD79A, DSC3
9thyroiditis30.7HLA-DRB1, CD79A
10herpes gestationis30.7DST
11sarcoma30.6DSG1, EVPL, DSP, JUP
12connective tissue disease30.3CD79A, HLA-DRB1
13epidermolysis bullosa30.2DST, DSP
14keratosis30.2DSP, JUP
15pemphigus vulgaris30.0DST, DSG1, DSP, JUP, HLA-DRB1, DSG2
16leukemia30.0EVPL, HLA-DRB1, CD79A, PLAUR
17ulcerative colitis29.5HLA-DRB1, CD79A, JUP
18melanoma29.3HLA-DRB1, JUP, DST, DSG1, PLAUR
19paraneoplastic pemphigus11.1
20pemphigus vegetans10.9
21pemphigus erythematosus10.8
22herpetiform pemphigus10.6
23nonepidermolytic palmoplantar keratoderma10.5EVPL
24castleman's disease10.5
25psoriasis10.4
26lichen planus10.4
27bronchiolitis10.4
28bronchiolitis obliterans10.4
29myasthenia gravis10.4
30lupus erythematosus10.4
31dendritic cell sarcoma10.4
32dermatitis10.4
33pemphigus and fogo selvagem10.4
34follicular dendritic cell sarcoma10.4
35keratoderma10.4DSG1, DSP
36hodgkin's lymphoma10.3
37chronic lymphocytic leukemia10.3
38b-cell lymphomas10.3
39systemic lupus erythematosus10.3
40usher syndrome10.3
41esophagitis10.3
42cicatricial pemphigoid10.3
43bullous skin disease10.2DST, EVPL, DSG1
44paronychia10.2
45gingivitis10.2
46bullous impetigo10.2
47epidermolysis bullosa acquisita10.2DSG1, DST, CD79A
48alopecia10.2
49arthritis10.2
50follicular lymphoma10.2

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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Drug clinical trials:

Search ClinicalTrials for Pemphigus

Search NIH Clinical Center for Pemphigus

Inferred drug relations via UMLS62/NDF-RT40:

Genetic Tests for Pemphigus

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Anatomical Context for Pemphigus

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MalaCards organs/tissues related to Pemphigus:

32
Skin, Testes, T cells, B cells, Eye, Neutrophil, Lung, Thyroid, Tongue, Bone, Colon, Bone marrow, Lymph node, Thymus, Prostate, Bronchial epithelium, Kidney, Myeloid

Animal Models for Pemphigus or affiliated genes

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MGI Mouse Phenotypes related to Pemphigus:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053787.6PLAUR, DSG4, DST, DSP, JUP, ATP2C1
2MP:00053847.4ATP2C1, JUP, DSP, DSG2, PLAUR, CD79A
3MP:00107717.3EVPL, ATP2C1, JUP, DSP, DST, DSG4
4MP:00107687.1ATP2C1, JUP, DSP, DST, DSG4, DSG2

Publications for Pemphigus

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Articles related to Pemphigus:

(show top 50)    (show all 2006)
idTitleAuthorsYear
1
Paraneoplastic pemphigus associated with primary pulmonar follicular dendritic cell sarcoma showing good response to treatment. (25388966)
2014
2
Pemphigus. S2 Guideline for diagnosis and treatment - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). (25338479)
2014
3
Biomarkers of oxidative stress in epidermis of Tunisian pemphigus foliaceus patients. (22738420)
2013
4
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
5
Detection of autoantibodies to periplakin and envoplakin in paraneoplastic pemphigus but not idiopathic pulmonary fibrosis using full-length recombinant proteins. (24275244)
2013
6
Prognostic factors in pemphigus vulgaris and pemphigus foliaceus. (24102444)
2013
7
Signaling dependent and independent mechanisms in pemphigus vulgaris blister formation. (23226536)
2012
8
Pathogenic relevance of IgG and IgM antibodies against desmoglein 3 in blister formation in pemphigus vulgaris. (21718682)
2011
9
Acute zoster in known pemphigus vulgaris and bullous pemphigoid: avoiding the "disease flare" trap. (21571161)
2011
10
Paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome): clinical presentations and pathogenesis. (21605807)
2011
11
Prevalence and treatment of pemphigus in Iran. (21925018)
2011
12
Pemphigus vulgaris antigen mRNA quantification for the staging of sentinel lymph nodes in head and neck cancer. (19997107)
2010
13
The efficacy of nicotinamide gel 4% as an adjuvant therapy in the treatment of cutaneous erosions of pemphigus vulgaris. (20597951)
2010
14
Familial benign chronic pemphigus (Hailey-Hailey disease). (21163165)
2010
15
ABO blood groups and pemphigus vulgaris: no relationship. (20372776)
2010
16
Palm tissue displaying a polyclonal autoimmune response in patients affected by a new variant of endemic pemphigus foliaceus in Colombia, South America. (19889592)
2010
17
Binding of pemphigus vulgaris IgG to antigens in desmosome core domains excludes immune complexes rather than directly splitting desmosomes. (20222919)
2010
18
An imbalance in Akt/mTOR is involved in the apoptotic and acantholytic processes in a mouse model of pemphigus vulgaris. (19552768)
2009
19
Autoantibodies against desmocollins in European patients with pemphigus. (19456767)
2009
20
Loss of the desmosomal protein perp enhances the phenotypic effects of pemphigus vulgaris autoantibodies. (19158843)
2009
21
Antigen selection of anti-DSG1 autoantibodies during and before the onset of endemic pemphigus foliaceus. (19571823)
2009
22
Paraneoplastic pemphigus. (17683379)
2007
23
Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment. (17461818)
2007
24
Detection of pemphigus desmoglein 1 and desmoglein 3 autoantibodies and pemphigoid BP180 autoantibodies in saliva and comparison with serum values. (17026501)
2006
25
Direct immunofluorescence on uninvolved, lesional and perilesional skin in patients with endemic pemphigus foliaceus (fogo selvagem). (15567982)
2004
26
Mucosal dominant pemphigus vulgaris with anti-desmoplakin autoantibodies. (15243525)
2004
27
Exacerbation of paraneoplastic pemphigus by cyclophosphamide treatment: detection of novel autoantigens and bronchial autoantibodies. (15149520)
2004
28
Topical 0.1% tacrolimus for the treatment of discoid lupus erythematosus and pemphigus erythematosus in dogs. (14736903)
2004
29
Association between HLA-DRB1, DQB1 genes and pemphigus vulgaris in Chinese Hans]. (12579512)
2003
30
Commentary/millenium: endemic pemphigus foliaceus (fogo selvagem). (11123439)
2000
31
Paraneoplastic pemphigus with circulating antibodies directed exclusively against the pemphigus vulgaris antigen desmoglein 3. (11004638)
2000
32
Analysis of the T cells that are potentially involved in autoantibody production in pemphigus vulgaris. (10635617)
1999
33
Antibodies to desmoplakin in a patient with pemphigus foliaceous. (9784047)
1998
34
Diet and pemphigus. In pursuit of exogenous factors in pemphigus and fogo selvagem. (9828876)
1998
35
Pemphigus IgG induces expression of urokinase plasminogen activator receptor on the cell surface of cultured keratinocytes. (9347794)
1997
36
Childhood paraneoplastic pemphigus associated with Castleman's tumour. (9039308)
1997
37
Mucosal and mucocutaneous (generalized) pemphigus vulgaris show distinct autoantibody profiles. (9326396)
1997
38
Herpetiform pemphigus showing reactivity with pemphigus vulgaris antigen (desmoglein 3) (9274636)
1997
39
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
40
Pemphigus foliaceus and pemphigus vulgaris autoantibodies react with the extracellular domain of desmoglein-1. (7860995)
1995
41
Atypical pemphigus with concomitant IgG and IgA anti-intercellular autoantibodies associated with monoclonal IgA gammopathy. (8049548)
1994
42
Pemphigus foliaceus treated successfully with plasma exchange. (1478768)
1992
43
Ultrastructural binding site of pemphigus foliaceus autoantibodies: comparison with pemphigus vulgaris. (1918503)
1991
44
Pemphigus vegetans presenting with deafness, otalgia and facial nerve paralysis. (2064948)
1991
45
Pemphigus foliaceus with anti-intercellular and anti-basement membrane zone antibodies. Blocking immunofluorescence and Western immunoblotting studies. (3069506)
1988
46
Sequence analysis of the HLA-DR beta and HLA-DQ beta loci from three Pemphigus vulgaris patients. (3391803)
1988
47
A newly characterized HLA DQ beta allele associated with pemphigus vulgaris. (2894075)
1988
48
Pemphigus foliaceus coexisting with toxic, multinodular goiter. (3771047)
1986
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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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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Compounds for genes affiliated with Pemphigus

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Sources:
44Novoseek, 24HMDB, 11DrugBank, 50PharmGKB
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Compounds related to Pemphigus according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1ivig449.8CD79A, DSG1
212-o-tetradecanoylphorbol 13-acetate449.2PLAUR, DST, DSP
3sucrose44 24 1110.8PLAUR, DSG1, DSP, JUP
4paraffin448.5JUP, PLAUR, CD79A, DST
5oligonucleotide448.3DST, HLA-DRB1, JUP, PLAUR
6retinoic acid44 249.2JUP, DSP, DSG1, CD79A, PLAUR
7estrogen448.1JUP, CD79A, PLAUR, ATP2C1
8tyrosine447.8ATP2C1, PLAUR, HLA-DRB1, DSG2, DSP, JUP
9calcium44 50 24 119.6PLAUR, ATP2C1, JUP, DSP, DST, DSG4

GO Terms for genes affiliated with Pemphigus

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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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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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet