MCID: PMP005
MIFTS: 57

Pemphigus Vulgaris malady

Skin category

Summaries for Pemphigus Vulgaris

Sources:
43NIH Rare Diseases, 64Wikipedia, 47OMIM, 33MalaCards
See all sources

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NIH Rare Diseases:43 Pemphigus vulgaris is an autoimmune disorder that involves blistering of the skin and mucous membranes. it occurs almost exclusively in middle-aged or older people. many cases begin with blisters in the mouth, followed by skin blisters that may come and go. in most cases, the exact cause of pemphigus vulgaris is unknown. it has rarely been observed in multiple members of the same family. treatment is aimed at reducing symptoms and preventing complications. severe cases are treated similarly to severe burns. last updated: 3/16/2010

MalaCards: Pemphigus Vulgaris is related to pemphigus and pemphigus foliaceus, and has symptoms including abnormal scarring/cheloids/hypertrophic scars, urticaria and vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment. An important gene associated with Pemphigus Vulgaris is DSG1 (desmoglein 1), and among its related pathways are Rho Family GTPases and Hypertrophic cardiomyopathy (HCM). The compounds endotoxin and asulam have been mentioned in the context of this disorder. Affiliated tissues include bone marrow, lymph node and small intestine, and related mouse phenotypes are hematopoietic system and integument.

Wikipedia:64 Pemphigus vulgaris is a chronic blistering skin disease with skin lesions that are rarely pruritic, but... more...

Description from OMIM:47 169610

Aliases & Classifications for Pemphigus Vulgaris

Sources:
43NIH Rare Diseases, 45Novoseek, 49Orphanet, 47OMIM, 31LifeMap Discovery™, 61UMLS, 26ICD10 via Orphanet, 58SNOMED-CT via Orphanet, 62UMLS via Orphanet, 25ICD10
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
pemphigus vulgaris:
Inheritance: Sporadic; Prevalence: 1-5/10000; Age of onset: Adulthood


Aliases & Descriptions:

pemphigus vulgaris 43 45 49 47 31 61


External Ids:

OMIM47 169610
ICD10 via Orphanet26 L10.0
SNOMED-CT via Orphanet58 49420001
UMLS via Orphanet62 C0030809
ICD1025 L10.0

Related Diseases for Pemphigus Vulgaris

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases in the Pemphigus Vulgaris family:

pemphigus vulgaris, familial

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

(show top 50)    (show all 161)
idRelated DiseaseScoreTop Affiliating Genes
1pemphigus32.3EVPL, JUP, PPL, DSP, DSG4, DSG3
2pemphigus foliaceus31.6PPL, HLA-DRB1, IVL, DSG3, DSG1, DSG2
3bullous pemphigoid31.0EVPL, DSP, PPL
4pemphigus erythematosus30.9DSP, DSG3
5lichen planus30.7CXCL9, DSG3, IVL
6pemphigus vegetans30.6DSG1
7squamous cell carcinoma30.6JUP, PPL, IVL, DSP, DSG3, DSC3
8hailey-hailey disease30.5CDH3, DSP, IVL, JUP
9silicosis30.4IL1A, DSG1
10thymoma30.4EVPL, IL1A, DSP, PPL
11arthritis30.4HLA-DRB1, CIITA, CXCL9, IL1A, FCER2
12ocular cicatricial pemphigoid30.4CIITA
13bronchiolitis obliterans30.1PPL
14erythema multiforme30.1DSP
15bronchiolitis30.1CCL11, FCER2
16adenocarcinoma30.1JUP, HLA-G, IVL, DSG3, DSC3, CDH3
17systemic lupus erythematosus30.1TNFSF13B, HLA-DRB1, CIITA, FCER2
18paraneoplastic pemphigus10.7
19paronychia10.5
20mucous membrane pemphigoid10.5
21pemphigus vulgaris, familial10.4
22oral lichen planus10.4
23folliculitis10.3
24cicatricial pemphigoid10.3
25nocardiosis10.3
26kaposi's sarcoma10.3
27tufted hair folliculitis10.3
28sepsis10.3
29acanthosis nigricans10.3
30eczema herpeticum10.3
31alopecia10.3
32polymyositis10.3
33herpetiform pemphigus10.3
34superficial pemphigus10.3
35hematopoietic stem cell transplantation10.3
36strongyloidiasis10.1
37cryoglobulinemia10.1
38hyperprolactinemia10.1
39cellulitis10.1
40brugada syndrome10.1
41aseptic meningitis10.1
42oral submucous fibrosis10.1
43gastric ulcer10.1
44localized scleroderma10.1
45infectious mononucleosis10.1
46secondary syphilis10.1
47listeria meningitis10.1
48cryptococcosis10.1
49porokeratosis10.1
50vitiligo10.1

Graphical network of the top 20 diseases related to Pemphigus Vulgaris:



Diseases related to pemphigus vulgaris

Clinical Features for Pemphigus Vulgaris

Sources:
47OMIM, 49Orphanet
See all sources

Clinical features from OMIM:

169610

Clinical synopsis from OMIM:

169610

Symptoms:

49 (show all 10)
  • abnormal scarring/cheloids/hypertrophic scars
  • urticaria
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • positive nikolski's sign/achantolysis
  • furuncle/cutaneous abscess/hidrosadenitis suppurativa
  • enanthema/aphtosa/aphta/leukoplakia
  • feeding disorder/dysphagia/swallowing/sucking disorder/esophageal dyskinesia
  • immunodeficiency/increased susceptibility to infections/recurrent infections
  • autoimmunity/autoimmune reaction/autoantibodies
  • weight loss/loss of appetite/break in weight curve/general health alteration

Drugs & Therapeutics for Pemphigus Vulgaris

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 31LifeMap Discovery™, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Pemphigus Vulgaris

Drug clinical trials:

Search ClinicalTrials for Pemphigus Vulgaris

Search NIH Clinical Center for Pemphigus Vulgaris

Search CenterWatch for Pemphigus Vulgaris

Cell-based therapeutics:


LifeMap Discovery
The database of embryonic development, stem cell research and regenerative medicine
Read about Pemphigus Vulgaris cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Pemphigus Vulgaris:
Hematopoietic stem cells for pemphigus

Genetic Tests for Pemphigus Vulgaris

Anatomical Context for Pemphigus Vulgaris

Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Pemphigus Vulgaris:

33
Bone marrow, Lymph node, Small intestine, Colon, Kidney, Lung, Thyroid, Salivary gland, Adrenal gland, Skin, T cells, B cells, Tongue

Animal Models for Pemphigus Vulgaris or affiliated genes

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

MGI Mouse Phenotypes related to Pemphigus Vulgaris:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000539710.1CIITA, HLA-G, TNFSF13B, CCL11, DSG4, DSG3
2MP:001077110.0DSG4, DSG3, CDH3, EVPL, JUP
3MP:00053879.8FCER2, JUP, CCL11, CCL17, TNFSF13B, HLA-G

Publications for Pemphigus Vulgaris

Sources:
51PubMed
See all sources

Articles related to Pemphigus Vulgaris:

(show top 50)    (show all 733)
idTitleAuthorsYear
1
Laboratory diagnosis of herpesvirus infections in patients with Pemphigus vulgaris lesions. (23735576)
2013
2
Plakophilin-1 Protects Keratinocytes from Pemphigus Vulgaris IgG by Forming Calcium-Independent Desmosomes. (24056861)
2013
3
Proposal for recognition of the comfort pattern in clients with pemphigus vulgaris using Fuzzy Logic. (24310696)
2013
4
Implant-supported oral rehabilitation of a patient with pemphigus vulgaris: a clinical report. (23552022)
2013
5
Oral hygiene in patients with gingival pemphigus vulgaris: a case series. (24353783)
2013
6
High levels of anti-heat shock protein 27 antibody in pemphigus vulgaris. (23717032)
2013
7
Skin tumors and skin infections in kidney transplant recipients vs. patients with pemphigus vulgaris. (23968536)
2013
8
Scaring alopecia in pemphigus vulgaris: a rare or underdiagnosed presentation? (23557714)
2013
9
Pemphigus vulgaris-associated Kaposi's sarcoma: response to paclitaxel and review of the literature. (24341453)
2013
10
Severe multi-resistant pemphigus vulgaris: prolonged remission with a single cycle of rituximab. (24068143)
2013
11
A case of cutaneous type pemphigus vulgaris. (21790548)
2012
12
Population-specific association between a polymorphic variant in ST18, encoding a pro-apoptotic molecule, and pemphigus vulgaris. (22437316)
2012
13
Case of juvenile pemphigus vulgaris which responded to i.v. immunoglobulin therapy. (21955154)
2012
14
Sweet syndrome and pemphigus vulgaris. (22513066)
2012
15
Non-junctional human desmoglein 3 acts as an upstream regulator of Src in E-cadherin adhesion, a pathway possibly involved in the pathogenesis of pemphigus vulgaris. (22294297)
2012
16
Pemphigus vulgaris: bilateral plica semilunaris involvement. (21045669)
2011
17
Unilesional pemphigus vulgaris of the scalp after cochlear implantation. (20015813)
2011
18
Desmosome disassembly in response to pemphigus vulgaris IgG occurs in distinct phases and can be reversed by expression of exogenous Dsg3. (21160493)
2011
19
Pemphigus vulgaris antigen mRNA quantification for the staging of sentinel lymph nodes in head and neck cancer. (19997107)
2010
20
TNF-alpha and IL-10 gene polymorphisms show a weak association with pemphigus vulgaris in the Slovak population. (19470040)
2010
21
Pemphigus vulgaris affecting 19 nails. (18795937)
2009
22
Secondary syphilis mimicking pemphigus vulgaris. (18721217)
2009
23
Pemphigus vulgaris with circulating anti-desmoglein 3 and anti-BP180 antibodies: a case report and brief review of cases with coexistence of pemphigus vulgaris and bullous pemphigoid. (19828097)
2009
24
CD28 T-cell costimulatory molecule expression in pemphigus vulgaris. (19207651)
2009
25
Severe pemphigus vulgaris: successful combination therapy of plasmapheresis followed by intravenous high-dose immunoglobulin to prevent rebound increase in pathogenic IgG. (18693160)
2008
26
The management of oral pemphigus vulgaris with systemic corticosteroid and dapsone. (23285328)
2008
27
Evidence of key role of Cdk2 overexpression in pemphigus vulgaris. (18199752)
2008
28
Effect of intravenous immunoglobulin therapy on serum levels of IgG1 and IgG4 antidesmoglein 1 and antidesmoglein 3 antibodies in pemphigus vulgaris. (19075146)
2008
29
Pemphigus vulgaris as a possible cause of protein-losing gastroenteropathy: a case report. (18307420)
2008
30
CD4+CD25high regulatory T cells are markedly decreased in blood of patients with pemphigus vulgaris. (17377382)
2007
31
Upregulation of c-Myc may contribute to the pathogenesis of canine pemphigus vulgaris. (17222234)
2007
32
Ear, nose and throat manifestations in pemphigus vulgaris. (17493073)
2007
33
Searching for experimental models of Pemphigus vulgaris. (17377799)
2007
34
Unusual thrombotic cardiac complications of Pemphigus vulgaris: a new link? (17186391)
2007
35
Nuclear c-Myc: a molecular marker for early stage pemphigus vulgaris. (17315040)
2007
36
Proteomic definition of a desmoglein linear determinant common to Pemphigus vulgaris and Pemphigus foliaceous. (16925820)
2006
37
Coexisting pemphigus vulgaris and pemphigus foliaceus in the same patient. (15689215)
2005
38
Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil. (12810504)
2003
39
Dichotomy of autoreactive Th1 and Th2 cell responses to desmoglein 3 in patients with pemphigus vulgaris (PV) and healthy carriers of PV-associated HLA class II alleles. (12496453)
2003
40
Cloning of canine desmoglein 3 and immunoreactivity of serum antibodies in human and canine pemphigus vulgaris with its extracellular domains. (14507443)
2003
41
Development of pemphigus vulgaris in a patient with pemphigus foliaceus: antidesmoglein antibody profile shift confirmed by enzyme-linked immunosorbent assay. (10767686)
2000
42
Analysis of the T cells that are potentially involved in autoantibody production in pemphigus vulgaris. (10635617)
1999
43
In vitro C3 mRNA expression in Pemphigus vulgaris: complement activation is increased by IL-1alpha and TNF-alpha. (10082594)
1999
44
Ultrastructural localization of cell junctional components (desmoglein, plakoglobin, E-cadherin, and beta-catenin) in Hailey-Hailey disease, Darier's disease, and pemphigus vulgaris. (9521500)
1998
45
Identification of a new antibody population directed against a desmosomal plaque antigen in pemphigus vulgaris and pemphigus foliaceus. (9077476)
1997
46
The location of binding sites of pemphigus vulgaris and pemphigus foliaceus autoantibodies: a post-embedding immunoelectron microscopic study. (9217820)
1997
47
Bilateral herpes simplex virus keratitis in a patient with pemphigus vulgaris. (8959902)
1996
48
Coexistence of psoriasis vulgaris and pemphigus foliaceus. (2620759)
1989
49
Coexistence of pemphigus vulgaris and progressive localized scleroderma. (2817919)
1989
50
Identification of pemphigus vulgaris antigen extracted from normal human epidermis and comparison with pemphigus foliaceus antigen. (3343340)
1988

Genetic Variations for Pemphigus Vulgaris

Expression for genes affiliated with Pemphigus Vulgaris

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Pemphigus Vulgaris

Search GEO for disease gene expression data for Pemphigus Vulgaris.

Pathways for genes affiliated with Pemphigus Vulgaris

Sources:
52QIAGEN, 30KEGG, 38NCBI BioSystems Database, 4Cell Signaling Technology, 12EMD Millipore, 54Reactome
See all sources

Pathways related to Pemphigus Vulgaris according to GeneCards/GeneDecks:

(show all 17)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.4CCL17, CDH3
2
Hide members
10.3DSG2, JUP, DSP
310.3DSP, CDH3, JUP
410.3EVPL, PPL, DSP
510.3HLA-DRB1, IL1A, TNFSF13B
610.3CCL11, FCER2, CCL17
710.3CCL11, CXCL9, CCL17
810.3IL1A, FCER2, HLA-DRB1
9
Hide members
10.3DSG2, DSG1, DSG3, DSP
1010.3DSG3, DSP, DSG2, DSG1
11
Hide members
10.3CIITA, HLA-DRB1, HLA-G, IL1A
12
Hide members
10.3HLA-G, IL1A, CIITA, HLA-DRB1
13
Hide members
10.2DSG3, DSG1, DSG2, DSP, HLA-G
14
Hide members
10.2CCL17, TNFSF13B, CXCL9, IL1A, CCL11
15
Hide members
10.2TNFSF13B, IL1A, CXCL9, CCL17, CCL11
16
Hide members
10.2CCL11, HLA-DRB1, HLA-G, CIITA, IL1A, TNFSF13B
17
Hide members
10.2CCL17, CXCL9, JUP, IL1A, TNFSF13B, CCL11

Compounds for genes affiliated with Pemphigus Vulgaris

Sources:
45Novoseek, 29IUPHAR, 11DrugBank, 24HMDB, 50PharmGKB, 60Tocris Bioscience
See all sources

Compounds related to Pemphigus Vulgaris according to GeneCards/GeneDecks:

(show all 33)
idCompoundScoreTop Affiliating Genes
1endotoxin4510.8CIITA
2asulam4510.7DSP, PPL
3pge24510.7CCL11
4roxithromycin45 29 1112.7IL1A, CCL17, CCL11
5rantes4510.7CCL11, CIITA, IL1A
6groalpha4510.6CCL11, CXCL9, IL1A
7tarc4510.6CCL11, CCL17, CXCL9, IL1A
8ccl345 2911.6CCL11, CCL17, CXCL9, IL1A
9il 104510.6IL1A, CXCL9, CCL17
10sucrose45 11 2412.6DSG1, DSP, CIITA, JUP
11ivig4510.6DSG3, DSG1, IL1A
12ionomycin4510.6CCL11, CXCL9, IL1A, FCER2
13dexamethasone45 50 29 1113.6JUP, CCL11, CCL17, CIITA, CXCL9, FCER2
14toxin a4510.6DSG1, DSG3
15allergens4510.6CCL11, CCL17, HLA-DRB1, CIITA, IVL, FCER2
16ccl445 2911.6CXCL9, CCL17, CCL11
17neopterin4510.5FCER2, IL1A, HLA-G
18thymidine45 2411.5CCL11, TNFSF13B, CIITA, IVL, IL1A, FCER2
19cyclosporin a45 29 6012.5CCL11, CCL17, CIITA, IVL, IL1A
20retinoic acid45 2411.5JUP, CCL11, IVL, DSP, DSG1, IL1A
21nitric oxide45 11 2412.5CCL11, CCL17, CIITA, CDH3, CXCL9, IL1A
2212-o-tetradecanoylphorbol 13-acetate4510.5IVL, DSP, IL1A, FCER2
23vegf4510.4JUP, CCL11, HLA-G, IVL, CXCL9, IL1A
24leukotriene b445 60 2412.4CCL11, IL1A, FCER2
25serine4510.4JUP, CIITA, IVL, DSP, DSG3, DSG1
26sb 20358045 6011.4FCER2, IL1A, IVL, CCL11
27histamine45 29 2412.3FCER2, CXCL9, CCL11, JUP
28sodium dodecylsulfate4510.3CIITA, IVL, IL1A
29tyrosine4510.3JUP, CCL11, HLA-DRB1, CIITA, IVL, DSP
30glatiramer acetate45 1111.3HLA-DRB1, CIITA
31oligonucleotide4510.2IVL, CIITA, HLA-G, HLA-DRB1, JUP
32calcium45 50 11 2413.1FCER2, JUP, CCL11, CCL17, TNFSF13B, HLA-G
33gold sodium thiomalate4510.1HLA-DRB1, IL1A

GO Terms for genes affiliated with Pemphigus Vulgaris

Sources:
16Gene Ontology
See all sources

Cellular components related to Pemphigus Vulgaris according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cell-cell junctionGO:00591110.3JUP, DSC3, DSG1, DSG2
2desmosomeGO:03005710.1JUP, EVPL, DSG2, DSG1, DSC3, DSG3
3cornified envelopeGO:00153310.0IVL, DSP, EVPL
4plasma membraneGO:0058869.8JUP, TNFSF13B, PPL, HLA-DRB1, HLA-G, FCER2

Biological processes related to Pemphigus Vulgaris according to GeneCards/GeneDecks:

(show all 16)
idNameGO IDScoreTop Affiliating Genes
1bundle of His cell to Purkinje myocyte communicationGO:08606910.6JUP, DSP, DSG2
2regulation of ventricular cardiac muscle cell action potentialGO:08600510.6DSG2, DSP, JUP
3regulation of heart rate by cardiac conductionGO:08609110.6JUP, DSP, DSG2
4cellular component disassembly involved in execution phase of apoptosisGO:00692110.6DSG2, DSG1, DSG3, DSP
5keratinocyte differentiationGO:03021610.5IVL, DSP, DSG4, EVPL
6peptide cross-linkingGO:01814910.5IVL, DSP, EVPL
7adherens junction organizationGO:03433210.5CDH3, DSP, JUP
8homophilic cell adhesionGO:00715610.5CDH3, DSG2, DSG1, DSC3, DSG3, DSG4
9keratinizationGO:03142410.5PPL, IVL, EVPL
10immune responseGO:00695510.4CCL11, CCL17, TNFSF13B, HLA-DRB1, CIITA, CXCL9
11interferon-gamma-mediated signaling pathwayGO:06033310.4HLA-DRB1, HLA-G, CIITA
12cytokine-mediated signaling pathwayGO:01922110.4IL1A, CIITA, HLA-G, HLA-DRB1
13cell-cell adhesionGO:01633710.4DSG1, DSP, JUP
14chemotaxisGO:00693510.3CXCL9, CCL17, CCL11
15inflammatory responseGO:00695410.2CCL11, CCL17, CXCL9, IL1A
16apoptotic processGO:0069159.9DSP, DSG3, DSG1, DSG2, IL1A

Molecular functions related to Pemphigus Vulgaris according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1gamma-catenin bindingGO:04529510.4DSG1, DSC3
2chemokine activityGO:00800910.3CCL11, CXCL9, CCL17
3calcium ion bindingGO:00550910.3DSG4, DSG3, DSC3, DSG1, DSG2, CDH3
4protein binding, bridgingGO:03067410.2EVPL, DSP, IVL
5structural molecule activityGO:00519810.0EVPL, JUP, IVL, DSP

Products for genes affiliated with Pemphigus Vulgaris

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Pemphigus Vulgaris

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