MCID: PMP001
MIFTS: 63

Pemphigus malady

Skin diseases category

Summaries for Pemphigus

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42NIH Rare Diseases, 33MedlinePlus, 63Wikipedia, 32MalaCards
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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: Pemphigus, also known as pemphigus nos, is related to hailey-hailey disease and bullous pemphigoid. An important gene associated with Pemphigus is DSG1 (desmoglein 1), and among its related pathways are Apoptotic cleavage of cellular proteins and Apoptotic cleavage of cell adhesion proteins. The drugs auranofin and chloroquine and the compounds calcium and tyrosine have been mentioned in the context of this disorder. Affiliated tissues include skin, testes and t cells, and related mouse phenotypes are hematopoietic system and integument.

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:63 Pemphigus (/ˈpɛmfɪɡəs/ or /pɛmˈfaɪɡəs/) is a rare group of blistering autoimmune diseases that... more...

Aliases & Classifications for Pemphigus

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8Disease Ontology, 42NIH Rare Diseases, 44Novoseek, 33MedlinePlus, 60UMLS, 34MeSH, 56SNOMED-CT, 39NCIt, 27ICD9CM, 25ICD10
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Classifications:

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


Aliases & Descriptions:

pemphigus 8 42 44 33 60
pemphigus nos 8


External Ids:

Disease Ontology8 DOID:9182
MeSH34 D010392
NCIt39 C34909
ICD9CM27 694.4
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 262)
idRelated DiseaseScoreTop Affiliating Genes
1hailey-hailey disease30.9DSP, ATP2C1, IVL, CDH3, JUP
2bullous pemphigoid30.9DSP, DST, PPL, EVPL
3thymoma30.6EVPL, PPL, DSP
4lupus erythematosus30.6CD79A
5pemphigus vulgaris30.6DSG2, DSP, DSG4, IVL, PLAU, CDH3
6sarcoma30.6JUP, EVPL, DSG1, DSP, PPL
7lichen planus30.6DST, IVL, DSG3
8psoriasis30.5HLA-DRB1, IVL, DST, CDH3
9myasthenia gravis30.5TNFSF13B, HLA-DQB1
10bronchiolitis obliterans30.5PPL
11pemphigus foliaceus30.5CD79A, IVL, DSG1, DSG2, DSG3, DST
12dermatitis30.5DSG3, DSG1, IVL, CD79A
13subcorneal pustular dermatosis30.5CD79A, DSG1, DSC3
14squamous cell carcinoma30.5DSC3, JUP, PLAUR, PLAU, DSG3, DSG1
15skin disease30.4DSG3, ATP2C1, DSP, CDH3, CD79A, DSC3
16leukemia30.4PLAUR, HLA-DRB1, CD79A, IVL, EVPL
17impetigo30.4DSG4
18pemphigus erythematosus30.4DSP, DSG3
19pemphigus vegetans30.4DSG1
20follicular dendritic cell sarcoma30.3PPL
21systemic lupus erythematosus30.3TNFSF13B
22cicatricial pemphigoid30.3DST, CD79A
23thyroiditis30.2HLA-DRB1, CD79A
24lung cancer30.1PLAU, ATP2C1
25bullous skin disease30.0DSG1, DSG3, EVPL, DST
26silicosis30.0DSG1
27hepatocellular carcinoma30.0PLAUR, CDH3, JUP, PLAU, HLA-DRB1
28keratosis29.9JUP, IVL, DSP
29dermatitis herpetiformis29.9CD79A, PLAU, HLA-DQB1
30melanoma29.9CDH3, PLAU, DSG1, PLAUR, HLA-DRB1, JUP
31ritter's disease29.9DSG1
32herpes gestationis29.9DST
33prostate cancer29.9CDH3, JUP, PLAUR, PLAU
34conjunctivitis29.9CD79A
35adenocarcinoma29.6JUP, DSG3, PLAUR, DSC3, PLAU, IVL
36epidermolysis bullosa29.6DSP, DST
37cryoglobulinemia29.6CD79A
38glomerulonephritis29.6CD79A
39hypotrichosis29.6DSG4
40autoimmune hemolytic anemia29.6CD79A
41hepatitis b29.6CD79A, HLA-DRB1, PLAUR, HLA-DQB1
42bronchiolitis10.4
43dendritic cell sarcoma10.3
44hodgkin's lymphoma10.3
45pemphigus and fogo selvagem10.3
46herpetiform pemphigus10.3
47usher syndrome10.2
48chronic lymphocytic leukemia10.2
49esophagitis10.2
50paronychia10.2

Graphical network of the top 20 diseases related to Pemphigus:



Diseases related to pemphigus

Clinical Features for Pemphigus

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Drugs & Therapeutics for Pemphigus

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Inferred drug relations via UMLS60/NDF-RT40:

Genetic Tests for Pemphigus

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Anatomical Context for Pemphigus

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32MalaCards
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MalaCards organs/tissues related to Pemphigus:

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

Animal Models for Pemphigus or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Pemphigus:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000539710.6HLA-DQB1, DSG4, DSG3, DST, CD79A, PLAU
2MP:001077110.5EVPL, JUP, ATP2C1, DSG4, DSG3, DST
3MP:000538410.3JUP, TNFSF13B, HLA-DQB1, ATP2C1, DSP, DSG3

Publications for Pemphigus

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50PubMed
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Articles related to Pemphigus:

(show top 50)    (show all 1951)
idTitleAuthorsYear
1
Mechanisms of mitochondrial damage in keratinocytes by pemphigus vulgaris antibodies. (23599429)
2013
2
Four mild but refractory cases of pemphigus foliaceus successfully treated with intravenous immunoglobulin. (24127870)
2013
3
Pemphigus with thymoma improved by thymectomy: report of a case. (22855008)
2013
4
Scanning electron microscopy of acantholysis in pemphigus foliaceus. (23793219)
2013
5
Corticosteroid-induced hyperglycemia is increased 10-fold in patients with pemphigus. (22994671)
2012
6
Pathogenic anti-desmoglein 3 mAbs cloned from a paraneoplastic pemphigus patient by phage display. (22277944)
2012
7
Pemphigus in elderly adults: clinical presentation, treatment, and prognosis. (22690997)
2012
8
Colocalization of mucosal vitiligo and oral pemphigus vulgaris. (22199076)
2012
9
Mucocutaneous-type pemphigus vulgaris with anti-desmoplakin autoantibodies. (21524991)
2011
10
Paraneoplastic pemphigus with Castleman's disease and bronchiolitis obliterans. (22181577)
2011
11
IgA pemphigus. (21679872)
2011
12
Pemphigus vulgaris antigen mRNA quantification for the staging of sentinel lymph nodes in head and neck cancer. (19997107)
2010
13
Pemphigus vulgaris of the pompholyx type. (20403799)
2010
14
Early invasive vulvar squamous cell carcinoma arising in a woman with vulvar pemphigus vulgaris and systemic lupus erythematosus. (20573220)
2010
15
Absence of human herpesvirus 8 in pemphigus and bullous pemphigoid. (19294004)
2009
16
In vivo confocal laser scanning microscopy for non-invasive diagnosis of pemphigus foliaceus. (19152577)
2009
17
Response of ocular pemphigus vulgaris to therapy. Case report and review of literature. (21886701)
2008
18
Novel system evaluating in vivo pathogenicity of desmoglein 3-reactive T cell clones using murine pemphigus vulgaris. (18606708)
2008
19
Differential coupling of M1 muscarinic and alpha7 nicotinic receptors to inhibition of pemphigus acantholysis. (18073210)
2008
20
Candida smear-an adjunct for diagnosis of acantholytic cells in oral pemphigus vulgaris. (18760058)
2008
21
Multiple autoimmune syndrome in a patient with pemphigus vulgaris: a new combination. (19216455)
2008
22
Paraneoplastic pemphigus associated with Castleman's disease: usefulness of the laboratory of autoimmunity in the diagnosis of this disease. (17804551)
2007
23
Paraneoplastic pemphigus associated with fludarabine use. (17673821)
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
How does acantholysis occur in pemphigus vulgaris: a critical review. (16776715)
2006
26
Simultaneous onset of pemphigus vulgaris in two Turkish siblings. (15863864)
2005
27
Beneficial effects of topical tacrolimus on recalcitrant erosions of pemphigus vulgaris. (15115509)
2004
28
IgA pemphigus of the subcorneal pustular dermatosis type associated with monoclonal IgA gammopathy. (14761150)
2003
29
The plant lectin wheat germ agglutinin inhibits the binding of pemphigus foliaceus autoantibodies to desmoglein 1 in a majority of patients and prevents pathomechanisms of pemphigus foliaceus in vitro and in vivo. (14634141)
2003
30
Protease inhibitors prevent plasminogen-mediated, but not pemphigus vulgaris-induced, acantholysis in human epidermis. (12675525)
2003
31
The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. Cooperative Group on Fogo Selvagem Research. (10882765)
2000
32
UVB-induced acantholysis in endemic Pemphigus foliaceus (Fogo selvagem) and Pemphigus vulgaris. (10727300)
2000
33
Human desmocollin 1 (Dsc1) is an autoantigen for the subcorneal pustular dermatosis type of IgA pemphigus. (9242496)
1997
34
Characterization of autoantibodies in pemphigus using antigen-specific enzyme-linked immunosorbent assays with baculovirus-expressed recombinant desmogleins. (9257868)
1997
35
A progressive blistering eruption in a patient with lymphoma. Paraneoplastic pemphigus. (9006379)
1997
36
Paronychia--a manifestation of pemphigus vulgaris. (8959911)
1996
37
Pemphigus foliaceus and pemphigus vulgaris autoantibodies react with the extracellular domain of desmoglein-1. (7860995)
1995
38
Plakoglobin binding by human Dsg3 (pemphigus vulgaris antigen) in keratinocytes requires the cadherin-like intracytoplasmic segment. (7738346)
1995
39
A simple epithelial cell line (MDCK) shows heterogeneity of desmoglein isoforms, one resembling pemphigus vulgaris antigen. (7615689)
1995
40
Comparative epidemiology of pemphigus in Tunisia and France: unusual incidence of pemphigus foliaceus in young Tunisian women. (7829889)
1995
41
Structure of DSG1, the bovine desmosomal cadherin gene encoding the pemphigus foliaceus antigen. Evidence of polymorphism. (8294446)
1994
42
Detection of anti-desmocollins I and II autoantibodies in two cases of Hallopeau type pemphigus vegetans by immunoblot analysis. (8060912)
1994
43
Histologic features of paraneoplastic pemphigus. (1497365)
1992
44
Pregnant women with endemic pemphigus foliaceus (Fogo Selvagem) give birth to disease-free babies. (1607681)
1992
45
The role of cyclosporine A in the treatment of pemphigus erythematosus. (1816136)
1991
46
A vegetating variety of pemphigus foliaceus. (2178988)
1990
47
Familial benign chronic pemphigus (Hailey-Hailey disease) and contact allergies. (2145330)
1990
48
Pemphigus vegetans in an unusual site. (2753595)
1989
49
Pemphigus erythematosus and heroin addiction. (659032)
1978
50
Pemphigus-like antibodies in cicatricial pemphigoid. (4592565)
1974

Genetic Variations for Pemphigus

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Expression for genes affiliated with Pemphigus

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1BioGPS, 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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53Reactome, 12EMD Millipore, 4Cell Signaling Technology, 29KEGG, 37NCBI BioSystems Database
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Compounds for genes affiliated with Pemphigus

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44Novoseek, 49PharmGKB, 11DrugBank, 24HMDB, 28IUPHAR, 59Tocris Bioscience
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Compounds related to Pemphigus according to GeneCards/GeneDecks:

(show all 26)
idCompoundScoreTop Affiliating Genes
1calcium44 49 11 2414.0PLAUR, JUP, TNFSF13B, ATP2C1, IVL, DSP
2tyrosine4411.0JUP, HLA-DQB1, HLA-DRB1, ATP2C1, IVL, DSP
3paraffin4410.9JUP, IVL, DST, CDH3, CD79A, PLAU
4retinoic acid44 2411.9JUP, IVL, DSP, DSG1, CD79A, PLAU
5estrogen4410.9JUP, ATP2C1, IVL, CDH3, CD79A, PLAU
612-o-tetradecanoylphorbol 13-acetate4410.9IVL, DSP, DST, PLAU, PLAUR
7oligonucleotide4410.9JUP, HLA-DQB1, HLA-DRB1, IVL, DST, PLAUR
8suramin44 28 1112.9PLAUR, PLAU, IVL
9sucrose44 11 2412.9JUP, DSP, DSG1, PLAUR
10asulam4410.8DSP, PPL
11thapsigargin44 5911.8PLAUR, PLAU, DSP, IVL
12ivig4410.8CD79A, DSG1, DSG3
13eaca4410.8PLAU, PLAUR
14rituximab44 49 1112.8CD79A, DSG1, TNFSF13B
15p-amino benzamidine4410.8PLAUR, PLAU
16allergens4410.8HLA-DRB1, IVL, CD79A
17pimonidazole4410.7PLAUR, IVL
18Urokinase1110.7PLAU, PLAUR
19saporin4410.7PLAU, PLAUR
20toxin a4410.7DSG1, DSG3
21blys4410.6CD79A, TNFSF13B
22alpha-amanitin4410.6PLAU, PLAUR
23steroid4410.5PLAUR, PLAU, CD79A, DSP, IVL
24tranexamic acid44 1111.5PLAU, PLAUR
25sodium dodecylsulfate4410.4CD79A, IVL
26serine4410.3JUP, IVL, DSP, DSG3, DSG1, PLAU

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:

idNameGO IDScoreTop Affiliating Genes
1plasma membraneGO:00588610.8DSP, HLA-DRB1, HLA-DQB1, PPL, TNFSF13B, JUP
2desmosomeGO:03005710.8EVPL, JUP, PPL, DSP, DSG4, DSG3
3integral to membraneGO:01602110.6DSG3, DSG4, ATP2C1, TNFSF13B, DST, DSC3
4cornified envelopeGO:00153310.6IVL, DSP, EVPL
5cell-cell junctionGO:00591110.6DSG2, DSG1, DSC3, JUP
6MHC class II protein complexGO:04261310.5HLA-DRB1, HLA-DQB1
7hemidesmosomeGO:03005610.4JUP, DST
8intermediate filamentGO:00588210.2DST, DSP, JUP

Biological processes related to Pemphigus according to GeneCards/GeneDecks:

(show all 14)
idNameGO IDScoreTop Affiliating Genes
1homophilic cell adhesionGO:00715610.8CDH3, DSG4, DSG3, DSC3, DSG1, DSG2
2cellular component disassembly involved in execution phase of apoptosisGO:00692110.8DSP, DSG3, DSG1, DSG2
3bundle of His cell to Purkinje myocyte communicationGO:08606910.8DSG2, DSP, JUP
4keratinocyte differentiationGO:03021610.8EVPL, DSG4, DSP, IVL
5regulation of ventricular cardiac muscle cell action potentialGO:08600510.8JUP, DSP, DSG2
6regulation of heart rate by cardiac conductionGO:08609110.7JUP, DSP, DSG2
7peptide cross-linkingGO:01814910.7IVL, DSP, EVPL
8adherens junction organizationGO:03433210.7CDH3, DSP, JUP
9keratinizationGO:03142410.6PPL, IVL, EVPL
10immunoglobulin production involved in immunoglobulin mediated immune responseGO:00238110.6HLA-DRB1, HLA-DQB1
11cell-cell adhesionGO:01633710.5DSG1, DSP, JUP
12T cell costimulationGO:03129510.5TNFSF13B, HLA-DQB1, HLA-DRB1
13humoral immune response mediated by circulating immunoglobulinGO:00245510.4HLA-DRB1, HLA-DQB1
14epidermis developmentGO:00854410.2EVPL, DSP, ATP2C1

Molecular functions related to Pemphigus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1calcium ion bindingGO:00550910.7CDH3, DSG2, DSG1, DSC3, DST, DSG3
2protein binding, bridgingGO:03067410.5IVL, DSP, EVPL
3gamma-catenin bindingGO:04529510.5DSG1, DSC3
4structural molecule activityGO:00519810.4EVPL, DSP, IVL, JUP
5MHC class II receptor activityGO:03239510.2HLA-DRB1, HLA-DQB1

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
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47OMIM via Orphanet
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57SNOMED-CT via Orphanet
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