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PF
MCID: PMP004
MIFTS: 43

Pemphigus Foliaceus (PF)

Categories: Rare diseases, Skin diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Pemphigus Foliaceus

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MalaCards integrated aliases for Pemphigus Foliaceus:

Name: Pemphigus Foliaceus 52 58 54 37 17 71 32
Pf 52

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Skin diseases
See all MalaCards categories (disease lists)
ICD10: 32 33
Orphanet: 58  
Rare skin diseases


External Ids:

ICD10 32 L10.2
ICD10 via Orphanet 33 L10.2
UMLS via Orphanet 72 C0263313
Orphanet 58 ORPHA79481
UMLS 71 C0263313
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Summaries for Pemphigus Foliaceus

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NIH Rare Diseases : 52 The following summary is from Orphanet , a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 79481 Definition A rare superficial pemphigus disease characterized by multiple, pruritic, scaly, crusted cutaneous erosions, with flaky circumscribed patches, localized mostly on the face, scalp, trunk and extremities, often presenting an erythematous base. Mucosal involvement is rarely observed. Visit the Orphanet disease page for more resources.

MalaCards based summary : Pemphigus Foliaceus, also known as pf, is related to pemphigus and bullous impetigo. An important gene associated with Pemphigus Foliaceus is DSG1 (Desmoglein 1), and among its related pathways/superpathways are Developmental Biology and Apoptotic execution phase. The drug Tyrosine has been mentioned in the context of this disorder. Affiliated tissues include skin, testes and b cells, and related phenotypes are abnormal blistering of the skin and erythema

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Related Diseases for Pemphigus Foliaceus

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Diseases related to Pemphigus Foliaceus via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 227)
# Related Disease Score Top Affiliating Genes
1 pemphigus 31.0 PPL HLA-DRB1 EVPL DST DSG3 DSG2
2 bullous impetigo 30.8 DSG3 DSG1
3 pemphigus vegetans 30.8 PPL DSG1
4 pyoderma 30.6 DSG3 DSG1
5 autoimmune disease 30.5 HLA-DRB1 DSG3 DSG1 CTLA4
6 impetigo 30.4 DSG3 DSG2 DSG1
7 benign chronic pemphigus 30.3 DSG3 DSG1
8 herpetiform pemphigus 30.1 DST DSG3 DSG1
9 iga pemphigus 30.1 PPL EVPL DSG3 DSG1
10 epidermolysis bullosa acquisita 29.9 HLA-DRB1 DST
11 lichen planus 29.9 DST DSG3 DSG1
12 thymoma 29.9 PPL EVPL CTLA4
13 ritter's disease 29.8 DSG3 DSG2 DSG1
14 skin disease 29.6 DST DSG3 DSG2 DSG1 CTLA4
15 bullous pemphigoid 29.4 PPL HLA-DRB1 EVPL DST DSG3 DSG1
16 pemphigus vulgaris, familial 29.3 PPL HLA-DRB1 EVPL DST DSG3 DSG2
17 subcorneal pustular dermatosis 28.7 PPL EVPL DST DSG3 DSG1
18 paraneoplastic pemphigus 28.6 PPL HLA-DRB1 EVPL DST DSG3 DSG2
19 bullous skin disease 28.4 PPL EVPL DST DSG3 DSG2 DSG1
20 cellulitis 28.2 EVPL DST DSG3 DSG1 CTLA4
21 pemphigus and fogo selvagem 12.0
22 pemphigus erythematosus 11.9
23 superficial pemphigus 11.1
24 pustulosis of palm and sole 10.7
25 psoriasis 10.7
26 alopecia 10.6
27 myasthenia gravis 10.6
28 dermatitis 10.6
29 thymoma, familial 10.5
30 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.5
31 retinitis pigmentosa-deafness syndrome 10.5
32 usher syndrome 10.5
33 dermatitis herpetiformis, familial 10.4
34 dermatitis herpetiformis 10.4
35 lupus erythematosus 10.4
36 systemic lupus erythematosus 10.3
37 exanthem 10.3
38 strongyloidiasis 10.3
39 vaccinia 10.3
40 herpes simplex 10.3
41 exfoliative dermatitis 10.3
42 acanthosis nigricans 10.3
43 kaposi sarcoma 10.3
44 diffuse alopecia areata 10.3
45 keratosis 10.3
46 squamous cell carcinoma 10.3
47 contact dermatitis 10.3
48 seborrheic dermatitis 10.3
49 cytokine deficiency 10.3
50 grover's disease 10.3

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



Diseases related to Pemphigus Foliaceus
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Symptoms & Phenotypes for Pemphigus Foliaceus

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Human phenotypes related to Pemphigus Foliaceus:

58 31 (show all 6)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abnormal blistering of the skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0008066
2 erythema 58 31 hallmark (90%) Very frequent (99-80%) HP:0010783
3 skin vesicle 58 31 hallmark (90%) Very frequent (99-80%) HP:0200037
4 skin erosion 58 31 hallmark (90%) Very frequent (99-80%) HP:0200041
5 acantholysis 58 31 hallmark (90%) Very frequent (99-80%) HP:0100792
6 crusting erythematous dermatitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0007473

MGI Mouse Phenotypes related to Pemphigus Foliaceus:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 9.1 CTLA4 DSG1 DSG2 DSG3 EVPL PPL
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Drugs & Therapeutics for Pemphigus Foliaceus

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Drugs for Pemphigus Foliaceus (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tyrosine Approved, Investigational, Nutraceutical Phase 3 60-18-4 6057

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Randomized, Double-Blind, Placebo-Controlled, Multicenter Trial to Evaluate the Efficacy and Safety of Oral BTK Inhibitor PRN1008 in Moderate to Severe Pemphigus Recruiting NCT03762265 Phase 3 PRN1008 Oral Tablet;Placebo Oral Tablet
2 A Phase I, Open-Label, Multicenter Trial Exploring the Safety and Tolerability of Autologous Polyclonal Regulatory T Cell Therapy in Adults With Active Pemphigus (APG01) Recruiting NCT03239470 Phase 1
3 Phenotypic and Functional Characterisation of Human B-cell Response in Pemphigus and Application to Other Auto-immune Diseases Recruiting NCT04117529
4 The Role of Trichoscopy in the Preliminary Diagnosis of Immunobollous Diseases Not yet recruiting NCT03478072

Search NIH Clinical Center for Pemphigus Foliaceus

Cell-based therapeutics:


LifeMap Discovery
Data from LifeMap, the Embryonic Development and Stem Cells Database
Read about Pemphigus Foliaceus cell therapies at LifeMap Discovery.
Stem-cell-based therapeutic approaches for Pemphigus Foliaceus:
Hematopoietic stem cells for pemphigus
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Genetic Tests for Pemphigus Foliaceus

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Anatomical Context for Pemphigus Foliaceus

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MalaCards organs/tissues related to Pemphigus Foliaceus:

40
Skin, Testes, B Cells, T Cells, Neutrophil, Thyroid, Breast
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Publications for Pemphigus Foliaceus

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Articles related to Pemphigus Foliaceus:

(show top 50) (show all 1387)
# Title Authors PMID Year
1
The enigmatic autoimmune response in endemic pemphigus foliaceus. 54 61
20096161 2009
2
Antigen selection of anti-DSG1 autoantibodies during and before the onset of endemic pemphigus foliaceus. 54 61
19571823 2009
3
Antibodies to the desmoglein 1 precursor proprotein but not to the mature cell surface protein cloned from individuals without pemphigus. 61 54
19843946 2009
4
Dermatoses affecting desmosomes in animals: a mechanistic review of acantholytic blistering skin diseases. 61 54
20178467 2009
5
Pathogenic epitopes of autoantibodies in pemphigus reside in the amino-terminal adhesive region of desmogleins which are unmasked by proteolytic processing of prosequence. 61 54
19340014 2009
6
Individual and epistatic effects of genetic polymorphisms of B-cell co-stimulatory molecules on susceptibility to pemphigus foliaceus. 61 54
19421221 2009
7
Apoptosis in pemphigus. 61 54
19189866 2009
8
Canine pemphigus foliaceus antigen is localized within desmosomes of keratinocyte. 54 61
18976819 2009
9
Advances in pemphigus and its endemic pemphigus foliaceus (Fogo Selvagem) phenotype: a paradigm of human autoimmunity. 61 54
18838249 2008
10
Anti-desmoglein IgG autoantibodies in patients with pemphigus in remission. 61 54
18410336 2008
11
E-cadherin is an additional immunological target for pemphigus autoantibodies. 61 54
18219275 2008
12
Expression of pemphigus-autoantigen desmoglein 1 in human thymus. 54 61
18331528 2008
13
Isolation of pathogenic monoclonal anti-desmoglein 1 human antibodies by phage display of pemphigus foliaceus autoantibodies. 54 61
18007588 2008
14
Pemphigus IgG causes skin splitting in the presence of both desmoglein 1 and desmoglein 3. 61 54
17640963 2007
15
An erythrodermic variant of pemphigus foliaceus with puzzling histologic and immunopathologic features. 61 54
17942028 2007
16
Internalization of non-clustered desmoglein 1 without depletion of desmoglein 1 from adhesion complexes in an experimental model of the autoimmune disease pemphigus foliaceus. 54 61
17624248 2007
17
Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. 54 61
17263817 2007
18
Changes in desmoglein 1 expression and subcellular localization in cultured keratinocytes subjected to anti-desmoglein 1 pemphigus autoimmunity. 61 54
17058228 2007
19
A truncated alternative spliced isoform of human desmoglein 1 contains a specific T cell epitope binding to the pemphigus foliaceus-associated HLA class II DRbeta1*0102 molecule. 61 54
17056584 2006
20
Wolfgang Bargmann-Preis 2006. Pemphigus foliaceus igG causes dissociation of desmoglein 1-containing junctions without blocking desmoglein 1 transinteraction. 61 54
17140142 2006
21
Non-pathogenic anti-desmoglein 3 IgG autoantibodies in Fogo Selvagem. 61 54
16912688 2006
22
Prevalence of anti-desmoglein-3 antibodies in endemic regions of Fogo selvagem in Brazil. 54 61
16763546 2006
23
Study of desmoglein 1 and 3 antibody levels in relation to disease severity in Indian patients with pemphigus. 61 54
16766834 2006
24
Antibodies against desmoglein 1 in healthy subjects in endemic and nonendemic areas of pemphigus foliaceus (fogo selvagem) in Peru. 54 61
16700787 2006
25
No evidence for association of the TP53 12139 and the BAX-248 polymorphisms with endemic pemphigus foliaceus (fogo selvagem). 61 54
16611260 2006
26
Desmoglein-1 is a minor autoantigen in dogs with pemphigus foliaceus. 54 61
16293316 2006
27
Prolonged clinical remission of patients with severe pemphigus upon rapid removal of desmoglein-reactive autoantibodies by immunoadsorption. 54 61
16484825 2006
28
Pemphigus foliaceus IgG causes dissociation of desmoglein 1-containing junctions without blocking desmoglein 1 transinteraction. 54 61
16211092 2005
29
Pemphigus foliaceus and desmoglein 1 gene polymorphism: is there any relationship? 54 61
16242304 2005
30
A comparison of anti-desmoglein antibodies and indirect immunofluorescence in the serodiagnosis of pemphigus vulgaris. 61 54
15985020 2005
31
Mycophenolate mofetil as an adjuvant therapy for classic and endemic pemphigus foliaceus. 54 61
16335874 2005
32
The interaction of pemphigus autoimmunoglobulins with epidermal cells: activation of the fas apoptotic pathway and the use of caspase activity for pathogenicity tests of pemphigus patients. 54 61
16014554 2005
33
Mechanisms of blister formation by staphylococcal toxins. 61 54
15671483 2004
34
Cytokine gene polymorphisms in endemic pemphigus foliaceus: a possible role for IL6 variants. 61 54
15566952 2004
35
Anti-desmoglein 1 antibodies in Tunisian healthy subjects: arguments for the role of environmental factors in the occurrence of Tunisian pemphigus foliaceus. 54 61
15196262 2004
36
A sensitive and restricted enzyme-linked immunosorbent assay for detecting a heterogeneous antibody population in serum from people suffering from a new variant of endemic pemphigus. 54 61
14730452 2004
37
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. 54 61
14634141 2003
38
Pathogenicity and epitope characteristics of anti-desmoglein-1 from pemphigus foliaceus patients expressing only IgG1 autoantibodies. 61 54
14675185 2003
39
Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia. 61 54
14512904 2003
40
Comparative study of autoantigen profile between Colombian and Brazilian types of endemic pemphigus foliaceus by various biochemical and molecular biological techniques. 54 61
12788527 2003
41
Recent advances in the study of the pathophysiology of pemphigus. 54 61
12677426 2003
42
Paraneoplastic pemphigus is associated with the DRB1*03 allele. 54 61
12604316 2003
43
The role of subclass switching in the pathogenesis of endemic pemphigus foliaceus. 54 61
12535205 2003
44
Influence of IVIg therapy on autoantibody titers to desmoglein 1 in patients with pemphigus foliaceus. 54 61
12482393 2002
45
Tunisian endemic pemphigus foliaceus is associated with desmoglein 1 gene polymorphism. 54 61
12209366 2002
46
T cell receptor Beta chain gene usage in endemic pemphigus foliaceus (fogo selvagem). 61 54
12190860 2002
47
Monitoring disease activity in pemphigus with enzyme-linked immunosorbent assay using recombinant desmogleins 1 and 3. 61 54
12174096 2002
48
A subset of pemphigus foliaceus patients exhibits pathogenic autoantibodies against both desmoglein-1 and desmoglein-3. 54 61
11982757 2002
49
Upregulation of P-cadherin expression in the lesional skin of pemphigus, Hailey-Hailey disease and Darier's disease. 54 61
11401672 2001
50
Increased antibody levels to desmogleins 1 and 3 after administration of carbamazepine. 61 54
11488835 2001
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Variations for Pemphigus Foliaceus

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Expression for Pemphigus Foliaceus

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Search GEO for disease gene expression data for Pemphigus Foliaceus.
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Pathways for Pemphigus Foliaceus

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Pathways related to Pemphigus Foliaceus according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Developmental Biology 65
Show member pathways
 12.77 PPL EVPL DSG3 DSG2 DSG1
2
Apoptotic execution phase 65
Show member pathways
 12.16 DSG3 DSG2 DSG1
3
Keratinization 65
Show member pathways
 11.48 PPL EVPL DSG3 DSG2 DSG1
4
Cytoskeleton remodeling Neurofilaments 22
Show member pathways
 11.17 PPL EVPL DST
5
CTLA4 Signaling 63
11.11 HLA-DRB1 CTLA4
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GO Terms for Pemphigus Foliaceus

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Cellular components related to Pemphigus Foliaceus according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 membrane GO:0016020 10.01 PPL HLA-DRB1 EVPL DST DSG3 DSG2
2 plasma membrane GO:0005886 9.91 PPL HLA-DRB1 DST DSG3 DSG2 DSG1
3 extracellular exosome GO:0070062 9.85 PPL HLA-DRB1 EVPL DSG3 DSG2
4 cell junction GO:0030054 9.63 PPL EVPL DST DSG3 DSG2 DSG1
5 intermediate filament GO:0005882 9.56 PPL HLA-DRB1 EVPL DST
6 cell-cell junction GO:0005911 9.5 DSG3 DSG2 DSG1
7 lateral plasma membrane GO:0016328 9.43 DSG2 DSG1
8 cornified envelope GO:0001533 9.35 PPL EVPL DSG3 DSG2 DSG1
9 desmosome GO:0030057 9.02 PPL EVPL DSG3 DSG2 DSG1

Biological processes related to Pemphigus Foliaceus according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 9.71 DST DSG3 DSG2 DSG1
2 cell-cell adhesion GO:0098609 9.54 DSG3 DSG2 DSG1
3 homophilic cell adhesion via plasma membrane adhesion molecules GO:0007156 9.5 DSG3 DSG2 DSG1
4 negative regulation of T cell proliferation GO:0042130 9.48 HLA-DRB1 CTLA4
5 response to progesterone GO:0032570 9.46 DSG2 DSG1
6 maternal process involved in female pregnancy GO:0060135 9.43 DSG2 DSG1
7 wound healing GO:0042060 9.43 PPL EVPL DST
8 keratinization GO:0031424 9.35 PPL EVPL DSG3 DSG2 DSG1
9 intermediate filament cytoskeleton organization GO:0045104 9.33 PPL EVPL DST
10 cornification GO:0070268 9.02 PPL EVPL DSG3 DSG2 DSG1

Molecular functions related to Pemphigus Foliaceus according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 calcium ion binding GO:0005509 9.26 DST DSG3 DSG2 DSG1
2 structural molecule activity GO:0005198 8.8 PPL EVPL DST
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Sources for Pemphigus Foliaceus

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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