MCID: PMP001
MIFTS: 60

Pemphigus malady

Skin category

Summaries for Pemphigus

Sources:
43NIH Rare Diseases, 34MedlinePlus, 64Wikipedia, 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 vulgaris and pemphigus foliaceus. 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, tongue and spinal cord, and related mouse phenotypes are hematopoietic system and integument.

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

Aliases & Classifications for Pemphigus

Sources:
8Disease Ontology, 43NIH Rare Diseases, 45Novoseek, 34MedlinePlus, 61UMLS, 35MeSH, 57SNOMED-CT, 40NCIt, 27ICD9CM, 25ICD10
See all sources

Classifications:

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


Aliases & Descriptions:

pemphigus 8 43 45 34 61
pemphigus nos 8


External Ids:

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

Related Diseases for Pemphigus

Sources:
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 234)
idRelated DiseaseScoreTop Affiliating Genes
1pemphigus vulgaris31.9EVPL, PLAUR, PLAU, JUP, TNFSF13B, PPL
2pemphigus foliaceus31.8PPL, HLA-DRB1, IVL, DSG3, DST, DSG1
3pemphigus vegetans31.3DSG1
4pemphigus erythematosus31.2DSP, DSG3
5hailey-hailey disease31.0JUP, ATP2C1, IVL, DSP, CDH3
6bullous pemphigoid31.0EVPL, PLAUR, PLAU, TNFSF13B, PPL, HLA-DQB1
7thymoma30.7EVPL, DSP, PPL
8lichen planus30.6DST, DSG3, IVL
9bronchiolitis obliterans30.6PPL
10subcorneal pustular dermatosis30.5CD79A, DSG1, DSC3
11squamous cell carcinoma30.5DSG3, DSP, IVL, PPL, JUP, DST
12follicular dendritic cell sarcoma30.4PPL
13systemic lupus erythematosus30.4TNFSF13B
14cicatricial pemphigoid30.3CD79A, DST
15bullous impetigo30.3DSG1
16silicosis30.1DSG1
17ritter's disease29.9DSG1
18herpes gestationis29.9DST
19adenocarcinoma29.7JUP, IVL, DSG3, DSC3, CDH3, PLAU
20cryoglobulinemia29.7CD79A
21autoimmune hemolytic anemia29.7CD79A
22paraneoplastic pemphigus11.1
23herpetiform pemphigus10.7
24superficial pemphigus10.4
25bronchiolitis10.4
26mucous membrane pemphigoid10.4
27usher syndrome10.3
28castleman's disease10.3
29pemphigus and fogo selvagem10.2
30follicular lymphoma10.2
31paronychia10.2
32oral lichen planus10.2
33acanthosis nigricans10.2
34kaposi's sarcoma10.2
35alopecia10.2
36stomatitis10.2
37lichen planus pemphigoides10.2
38folliculitis10.1
39arthritis10.1
40pemphigus vulgaris, familial10.1
41toxic epidermal necrolysis10.1
42eczema herpeticum10.1
43nocardiosis10.1
44porokeratosis10.1
45vascular disease10.1
46ocular cicatricial pemphigoid10.1
47transient acantholytic dermatosis10.1
48polymyositis10.1
49tufted hair folliculitis10.1
50sepsis10.1

Graphical network of the top 20 diseases related to Pemphigus:



Diseases related to pemphigus

Clinical Features for Pemphigus

Drugs & Therapeutics for Pemphigus

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
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Approved drugs:

Search CenterWatch for Pemphigus

Drug clinical trials:

Search ClinicalTrials for Pemphigus

Search NIH Clinical Center for Pemphigus

Search CenterWatch for Pemphigus

Inferred drug relations via UMLS61/NDF-RT41:

Genetic Tests for Pemphigus

Anatomical Context for Pemphigus

Sources:
33MalaCards
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MalaCards organs/tissues related to Pemphigus:

33
Skin, Tongue, Spinal cord, Kidney, Lymph node, Lung, Bone marrow, Thymus, Small intestine, Colon, Thyroid, Salivary gland, Adrenal gland, Prostate, Myeloid, T cells, B lymphoblasts, B cells, Bronchial epithelium

Animal Models for Pemphigus or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Pemphigus:

37
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

Sources:
51PubMed
See all sources

Articles related to Pemphigus:

(show top 50)    (show all 1952)
idTitleAuthorsYear
1
Biomarkers of oxidative stress in epidermis of Tunisian pemphigus foliaceus patients. (22738420)
2013
2
Study of apoptosis in oral pemphigus vulgaris using immunohistochemical marker Bax and TUNEL technique. (23186378)
2013
3
Interdigitating dendritic cell sarcoma complicated with paraneoplastic pemphigus after surgery of resection. (23652075)
2013
4
The course and prognosis of pemphigus in 47 Tunisian patients. (22145661)
2013
5
Recalcitrant pemphigus herpetiformis with high titer of immunoglobulin G antibody to desmoglein 1 and positive IgG antibody to desmocollin 3, elevating thymus and activation-regulated chemokine. (23432082)
2013
6
Overlap of IgA Pemphigus and Linear IgA Dermatosis in a Patient with Ulcerative Colitis: A Mere Coincidence? (23824334)
2013
7
A case of Paraneoplastic Pemphigus associated with Castleman's disease. (24346868)
2013
8
Treatment of severe pemphigus with a combination of immunoadsorption, rituximab, pulsed dexamethasone and azathioprine/mycophenolate mofetil: a pilot study of 23 patients. (21910700)
2012
9
Pemphigus foliaceus clearance status postmyomectomy for an enlarged infarcted subserous uterine fibroid. (22583728)
2012
10
Low-level laser therapy on the treatment of oral and cutaneous pemphigus vulgaris: case report. (22538841)
2012
11
Localized pemphigus with vegetative features. (23286801)
2012
12
Profile of Trypanosoma cruzi reactivity in a population at high risk for endemic pemphigus foliaceus (Fogo selvagem). (22826496)
2012
13
Transient acantholytic dermatosis associated with pemphigus foliaceus. (23067093)
2012
14
Two distinct viral infections complicating pemphigus foliaceus. (22301040)
2012
15
Inhibition of FAK prevents blister formation in the neonatal mouse model of pemphigus vulgaris. (22320676)
2012
16
Mental health in patients with pemphigus: an issue to worth consideration. (22121274)
2011
17
A unique case of intra-epidermal neutrophilic dermatosis-type IgA pemphigus presenting with subcorneal pustules. (21228550)
2011
18
Urgent referrals. Eczema herpeticum, pemphigus vulgaris, perinephric abscess, quinsy, necrotising fasciitis. (21510509)
2011
19
Marked elevation of serum macrophage migration inhibitory factor levels in patients with pemphigus vulgaris. (19799642)
2010
20
Canine pemphigus foliaceus antigen is localized within desmosomes of keratinocyte. (18976819)
2009
21
Nail unit involvement during severe initial pemphigus vulgaris development. (19258233)
2009
22
In situ detection of inflammatory cytokines and apoptosis in pemphigus foliaceus patients. (19123745)
2009
23
Metastatic prostate cancer presenting as paraneoplastic pemphigus: a favourable clinical response to combined androgen blockade and conventional immunosuppressive therapy. (19120335)
2009
24
Intravenous immunoglobulin therapy in two patients with myasthenia gravis and pemphigus vulgaris. (20476669)
2009
25
Simultaneous occurrence of anti-BP180 mucous membrane pemphigoid and mucosal-dominant pemphigus vulgaris. (19778312)
2009
26
Severe pemphigus vulgaris: successful combination therapy of plasmapheresis followed by intravenous high-dose immunoglobulin to prevent rebound increase in pathogenic IgG. (18693160)
2008
27
Expression of pemphigus-autoantigen desmoglein 1 in human thymus. (18331528)
2008
28
Polymorphism of the promoter region and exon 1 of the CTLA4 gene in endemic pemphigus foliaceus (fogo selvagem). (16972006)
2006
29
The N-terminal fraction of desmoglein 3 encompassing its immunodominant domain is present in human serum: implications for pemphigus vulgaris autoimmunity. (16831306)
2006
30
Value of direct immunofluorescence in predicting remission in pemphigus vulgaris. (17076711)
2006
31
Pemphigus in the XXI century: new life to an old story. (17135055)
2006
32
Successful treatment of subcorneal pustular type of IgA pemphigus]. (15580453)
2005
33
Recent advances in the study of the pathophysiology of pemphigus. (12677426)
2003
34
Transition of pemphigus vulgaris into pemphigus foliaceus confirmed by antidesmoglein ELISA profile. (12207776)
2002
35
Paraneoplastic pemphigus associated with Castleman's disease and asymptomatic bronchiolitis obliterans. (12095881)
2002
36
Clinical and serological transition from pemphigus vulgaris to pemphigus foliaceus demonstrated by desmoglein ELISA system. (11790172)
2002
37
Further support for a role for Th2-like cytokines in blister formation of pemphigus. (11161984)
2001
38
Indapamide-induced pemphigus foliaceus. (12243711)
2001
39
The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. (10025740)
1999
40
Pemphigus vegetans in association with human immunodeficiency virus. (10561053)
1999
41
Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans. (10459112)
1999
42
Antibodies to desmoplakin in a patient with pemphigus foliaceous. (9784047)
1998
43
Paraneoplastic pemphigus: report of a case. (8708152)
1996
44
Atypical pemphigus with concomitant IgG and IgA anti-intercellular autoantibodies associated with monoclonal IgA gammopathy. (8049548)
1994
45
Pemphigus: past, present and future. (7841551)
1994
46
A case of herpetiform pemphigus associated with lung cancer. (7962962)
1994
47
Human autoantibodies against desmoplakins in paraneoplastic pemphigus. (1601988)
1992
48
Alopecia areata and pemphigus foliaceus. (4008696)
1985
49
Pyoderma gangrenosum associated with transient acantholytic dermatosis (pemphigus erythematosus-like) and paraproteinemia. (6164226)
1981
50
Pemphigus erythematosus. (18146563)
1949

Genetic Variations for Pemphigus

Expression for genes affiliated with Pemphigus

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

Sources:
54Reactome, 12EMD Millipore, 4Cell Signaling Technology, 30KEGG, 38NCBI BioSystems Database
See all sources

Compounds for genes affiliated with Pemphigus

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 24HMDB, 29IUPHAR, 60Tocris Bioscience
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Compounds related to Pemphigus according to GeneCards/GeneDecks:

(show all 26)
idCompoundScoreTop Affiliating Genes
1calcium45 50 11 2414.0PLAUR, JUP, TNFSF13B, ATP2C1, IVL, DSP
2tyrosine4511.0JUP, HLA-DQB1, HLA-DRB1, ATP2C1, IVL, DSP
3paraffin4510.9JUP, IVL, DST, CDH3, CD79A, PLAU
4retinoic acid45 2411.9JUP, IVL, DSP, DSG1, CD79A, PLAU
5estrogen4510.9JUP, ATP2C1, IVL, CDH3, CD79A, PLAU
612-o-tetradecanoylphorbol 13-acetate4510.9IVL, DSP, DST, PLAU, PLAUR
7oligonucleotide4510.9JUP, HLA-DQB1, HLA-DRB1, IVL, DST, PLAUR
8suramin45 29 1112.9PLAUR, PLAU, IVL
9sucrose45 11 2412.9JUP, DSP, DSG1, PLAUR
10asulam4510.8DSP, PPL
11thapsigargin45 6011.8PLAUR, PLAU, DSP, IVL
12ivig4510.8CD79A, DSG1, DSG3
13eaca4510.8PLAU, PLAUR
14rituximab45 50 1112.8CD79A, DSG1, TNFSF13B
15p-amino benzamidine4510.8PLAUR, PLAU
16allergens4510.8HLA-DRB1, IVL, CD79A
17pimonidazole4510.7PLAUR, IVL
18Urokinase1110.7PLAU, PLAUR
19saporin4510.7PLAU, PLAUR
20toxin a4510.7DSG1, DSG3
21blys4510.6CD79A, TNFSF13B
22alpha-amanitin4510.6PLAU, PLAUR
23steroid4510.5PLAUR, PLAU, CD79A, DSP, IVL
24tranexamic acid45 1111.5PLAU, PLAUR
25sodium dodecylsulfate4510.4CD79A, IVL
26serine4510.3JUP, IVL, DSP, DSG3, DSG1, PLAU

GO Terms for genes affiliated with Pemphigus

Sources:
16Gene Ontology
See all sources

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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  • Antibodies

Sources for Pemphigus

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet