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 chloroquine and chloroquine phosphate 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
An unusual epiglottis synechiae managed with transoral robotic surgery in a patient with pemphigus vulgaris. (24036806)
2013
2
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
3
Selective Elevation of Antibodies to Desmoglein 1 during the Transition from Mucocutaneous to Cutaneous Type Pemphigus Vulgaris. (23717031)
2013
4
Oral Ulcers as an Initial Presentation of Juvenile Pemphigus: A Case Report. (24295781)
2013
5
Myasthenia gravis, Castleman disease, pemphigus, and anti-phospholipid syndrome. (23386221)
2013
6
Correlation of antibodies against desmogleins 1 and 3 with indirect immunofluorescence and disease status in a Greek population with pemphigus vulgaris. (22221266)
2013
7
High levels of anti-heat shock protein 27 antibody in pemphigus vulgaris. (23717032)
2013
8
p38 MAPK Signaling in Pemphigus: Implications for Skin Autoimmunity. (23936634)
2013
9
Varicella-zoster virus (VZV) and alpha 1 antitrypsin: a fatal outcome in a patient affected by endemic pemphigus foliaceus. (22715825)
2012
10
Topical sirolimus for oral pemphigus vulgaris: 3 unresponsive cases. (23062929)
2012
11
Atypical pemphigus with exclusively anti-desmocollin 3-specific IgG antibodies. (22735276)
2012
12
Adjuvant rituximab therapy of pemphigus: a single-center experience with 31 patients. (22710375)
2012
13
Pemphigus foliaceus in a juvenile Cashmere goat, and outcome after prednisolone and methylprednisolone therapy. (22654142)
2011
14
Binding of pemphigus vulgaris IgG to antigens in desmosome core domains excludes immune complexes rather than directly splitting desmosomes. (20222919)
2010
15
Antimitochondrial autoantibodies in pemphigus vulgaris: a missing link in disease pathophysiology. (20007702)
2010
16
p38MAPK signaling and desmoglein-3 internalization are linked events in pemphigus acantholysis. (20093368)
2010
17
Fatal paraneoplastic pemphigus associated with a mediastinal tumor. (19087213)
2009
18
Photodynamic therapy: a useful adjunct therapy for recalcitrant ulceration in pemphigus vulgaris. (19415812)
2009
19
Apoptosis in pemphigus. (19189866)
2009
20
Secondary syphilis mimicking pemphigus vulgaris. (18721217)
2009
21
Evidence of key role of Cdk2 overexpression in pemphigus vulgaris. (18199752)
2008
22
HLA-DRB1*0402 haplotypes without DQB1*0302 in Venezuelan patients with pemphigus vulgaris. (17389015)
2007
23
Using desmoglein 1 and 3 enzyme-linked immunosorbent assay as an adjunct diagnostic tool for pemphigus. (17339147)
2007
24
A case of herpetiform pemphigus with anti-desmoglein 3 IgG autoantibodies. (15187309)
2004
25
Management of pemphigus vulgaris during acute phase. (17642849)
2003
26
Polymorphisms within the tumor necrosis factor and lymphotoxin-alpha genes and endemic pemphigus foliaceus--are there any associations? (14617046)
2003
27
The humanized SCID mouse model to study HLA class II-linked autoimmunity to desmoglein 3 in pemphigus vulgaris. (11903226)
2002
28
Endemic Pemphigus foliaceus ("Fogo selvagem"): a series from the Northeastern region of the State of SALo Paulo, Brazil, 1973-1998. (11340476)
2001
29
Novel human alpha9 acetylcholine receptor regulating keratinocyte adhesion is targeted by Pemphigus vulgaris autoimmunity. (11021840)
2000
30
Subcorneal pustular dermatosis type of IgA pemphigus: demonstration of autoantibodies to desmocollin-1 and clinical review. (10886149)
2000
31
Paraneoplastic pemphigus: a case of long-term survival associated with systemic lupus erythematosus and polymyositis. (10449962)
1999
32
Paraneoplastic pemphigus with a pemphigus vegetans-like plaque as the only cutaneous manifestation. (9810918)
1998
33
Pemphigus vulgaris associated with transient acanthosis nigricans like lesion. (9769604)
1998
34
Childhood paraneoplastic pemphigus associated with Castleman's tumour. (9039308)
1997
35
Targeted disruption of the pemphigus vulgaris antigen (desmoglein 3) gene in mice causes loss of keratinocyte cell adhesion with a phenotype similar to pemphigus vulgaris. (9166409)
1997
36
Pemphigus foliaceus: preferential binding of IgG1 and C3 at the upper epidermis. (9068743)
1997
37
Systemic lupus erythematosus, pemphigus erythematosus, and thymoma in the same patient. (19078051)
1996
38
Pemphigus vulgaris in distant relatives of two families: association with major histocompatibility complex class II genes. (8759194)
1996
39
High-titer interferon-alpha antibodies in a patient with pemphigus foliaceus. (8889468)
1996
40
Three cases of pemphigus vegetans: induction by enalapril--association with internal malignancy. (8169014)
1994
41
Environmental risk factors in endemic pemphigus foliaceus (Fogo selvagem). "The Cooperative Group on Fogo Selvagem Research". (1593148)
1992
42
IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis. (1646833)
1991
43
Coexistence of psoriasis vulgaris and pemphigus foliaceus. (2620759)
1989
44
Sequence analysis of the HLA-DR beta and HLA-DQ beta loci from three Pemphigus vulgaris patients. (3391803)
1988
45
Malodorous intertriginous pustules and plaques. Pemphigus vegetans, Hallopeau type. (3545092)
1987
46
Pemphigus with characteristics of dermatitis herpetiformis. A long-term follow-up of five patients. (3539834)
1986
47
"Familial benign chronic pemphigus" by Hailey and Hailey, April 1939. Commentary: Hailey-Hailey disease, familial benign chronic pemphigus. (6753757)
1982
48
Pemphigus erythematosus-like rash in a patient on penicillamine. (851751)
1977
49
On the association of some dermatoses with South American pemphigus foliaceus. (4371031)
1974
50
Impetigo Herpetiformis; Pemphigus Vegetans? (19993514)
1947

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
46OMIM
47OMIM via Orphanet
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51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet