PF
MCID: PMP004
MIFTS: 45

Pemphigus Foliaceus (PF) malady

Rare diseases, Skin diseases categories
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Summaries for Pemphigus Foliaceus

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65Wikipedia, 33MalaCards
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Wikipedia:65 Pemphigus foliaceus is an autoimmune blistering disease of the skin with characteristic lesions that are... more...

MalaCards: Pemphigus Foliaceus, also known as PF, is related to pemphigus and pemphigus vulgaris, and has symptoms including positive nikolski's sign/achantolysis An important gene associated with Pemphigus Foliaceus is DSG1 (desmoglein 1), and among its related pathways are Apoptotic cleavage of cellular proteins and Staphylococcus aureus infection. The compounds ivig and rituximab have been mentioned in the context of this disorder. Affiliated tissues include skin, testes and t cells.

Aliases & Classifications for Pemphigus Foliaceus

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43NIH Rare Diseases, 45Novoseek, 49Orphanet, 31LifeMap Discovery™, 62UMLS, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet, 25ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases
Orphanet: 49 
Rare skin diseases


Aliases & Descriptions:

pemphigus foliaceus 43 45 49 31 62
pf 43


External Ids:

ICD10 via Orphanet26 L10.2
SNOMED-CT via Orphanet59 35154004
UMLS via Orphanet63 C0263313
ICD1025 L10.2

Related Diseases for Pemphigus Foliaceus

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Pemphigus Foliaceus:



Diseases related to pemphigus foliaceus

Symptoms for Pemphigus Foliaceus

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49Orphanet
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Symptoms:

49
  • positive nikolski's sign/achantolysis

Drugs & Therapeutics for Pemphigus Foliaceus

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 31LifeMap Discovery™, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Pemphigus Foliaceus

Search NIH Clinical Center for Pemphigus Foliaceus

Cell-based therapeutics:


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

Genetic Tests for Pemphigus Foliaceus

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Anatomical Context for Pemphigus Foliaceus

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33MalaCards
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MalaCards organs/tissues related to Pemphigus Foliaceus:

33
Skin, Testes, T cells, B cells, Lymph node, Lung, Thyroid, Prostate

Animal Models for Pemphigus Foliaceus or affiliated genes

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Publications for Pemphigus Foliaceus

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52PubMed
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Articles related to Pemphigus Foliaceus:

(show top 50)    (show all 497)
idTitleAuthorsYear
1
Biomarkers of oxidative stress in epidermis of Tunisian pemphigus foliaceus patients. (22738420)
2013
2
Both qualitative and quantitative genetic variation of MHC class II molecules may influence susceptibility to autoimmune diseases: the case of endemic pemphigus foliaceus. (23777927)
2013
3
Prognostic factors in pemphigus vulgaris and pemphigus foliaceus. (24102444)
2013
4
Direct immunofluorescence of the outer root sheath in anagen and telogen hair in pemphigus vulgaris and pemphigus foliaceus. (23713982)
2013
5
Pemphigus foliaceus clearance status postmyomectomy for an enlarged infarcted subserous uterine fibroid. (22583728)
2012
6
Palm tissue displaying a polyclonal autoimmune response in patients affected by a new variant of endemic pemphigus foliaceus in Colombia, South America. (19889592)
2010
7
Antigen selection of anti-DSG1 autoantibodies during and before the onset of endemic pemphigus foliaceus. (19571823)
2009
8
Canine pemphigus foliaceus antigen is localized within desmosomes of keratinocyte. (18976819)
2009
9
Anti-desmoglein 1 antibodies in healthy related and unrelated subjects and patients with pemphigus foliaceus in endemic and non-endemic areas from Tunisia. (19453789)
2009
10
Advances in pemphigus and its endemic pemphigus foliaceus (Fogo Selvagem) phenotype: a paradigm of human autoimmunity. (18838249)
2008
11
Tinea incognito hidden under apparently treatment-resistant pemphigus foliaceus. (18480931)
2008
12
Recalcitrant pemphigus foliaceus with Kaposi's varicelliform eruption: report of a fatal case. (18801107)
2008
13
Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment. (17461818)
2007
14
Pemphigus vulgaris and pemphigus foliaceus: similar prognosis? (17822493)
2007
15
Internalization of non-clustered desmoglein 1 without depletion of desmoglein 1 from adhesion complexes in an experimental model of the autoimmune disease pemphigus foliaceus. (17624248)
2007
16
No evidence for association of the TP53 12139 and the BAX-248 polymorphisms with endemic pemphigus foliaceus (fogo selvagem). (16611260)
2006
17
Increased activity of plasma and tissue kallikreins, plasma kininase II and salivary kallikrein in pemphigus foliaceus (fogo selvagem). (15840094)
2005
18
Eyelid skin involvement in pemphigus foliaceus. (16419424)
2005
19
Ex vivo analysis of desmoglein 1-responsive T-helper (Th) 1 and Th2 cells in patients with pemphigus foliaceus and healthy individuals. (16026580)
2005
20
Direct immunofluorescence on uninvolved, lesional and perilesional skin in patients with endemic pemphigus foliaceus (fogo selvagem). (15567982)
2004
21
Pemphigus foliaceus with neutrophilic spongiosis evolving to an atypical pemphigus phenotype. (15583603)
2004
22
Anti-desmoglein 1 antibodies in Tunisian healthy subjects: arguments for the role of environmental factors in the occurrence of Tunisian pemphigus foliaceus. (15196262)
2004
23
Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil. (12810504)
2003
24
Comparative study of autoantigen profile between Colombian and Brazilian types of endemic pemphigus foliaceus by various biochemical and molecular biological techniques. (12788527)
2003
25
Pemphigus foliaceus localized to the nose. (12950358)
2003
26
Cutaneous neosporosis during treatment of pemphigus foliaceus in a dog. (12220024)
2002
27
Endemic Pemphigus foliaceus ("Fogo selvagem"): a series from the Northeastern region of the State of SALo Paulo, Brazil, 1973-1998. (11340476)
2001
28
A polymorphic variant of the gene coding desmoglein 1, the target autoantigen of pemphigus foliaceus, is associated with the disease. (11294567)
2001
29
Thyroid gland tumour, pemphigus foliaceus and myasthenia gravis in the daughter of a woman with myasthenia gravis. (11678876)
2001
30
Commentary/millenium: endemic pemphigus foliaceus (fogo selvagem). (11123439)
2000
31
Pemphigus foliaceus associated with silicosis. (10951170)
2000
32
An interesting case of pemphigus foliaceus in a dog. (10065323)
1999
33
Pemphigus foliaceus induced by psoralen-UV-A. (9801700)
1998
34
Pemphigus foliaceus: an unusual clinical feature and case reports. (9466075)
1998
35
Pemphigus foliaceus and pemphigus vulgaris autoantibodies react with the extracellular domain of desmoglein-1. (7860995)
1995
36
Tests of inflammatory activity in endemic pemphigus foliaceus]. (8008917)
1994
37
Structure of DSG1, the bovine desmosomal cadherin gene encoding the pemphigus foliaceus antigen. Evidence of polymorphism. (8294446)
1994
38
Pemphigus foliaceus treated successfully with plasma exchange. (1478768)
1992
39
Ultrastructural binding site of pemphigus foliaceus autoantibodies: comparison with pemphigus vulgaris. (1918503)
1991
40
Complement activation in Brazilian pemphigus foliaceus. (2805387)
1989
41
Coexistence of psoriasis vulgaris and pemphigus foliaceus. (2620759)
1989
42
The pathogenic effect of IgG4 autoantibodies in endemic pemphigus foliaceus (fogo selvagem). (2654636)
1989
43
Pemphigus foliaceus antigen: characterization of a keratinocyte envelope associated pool and preparation of a soluble immunoreactive fragment. (2474034)
1989
44
Pemphigus foliaceus with anti-intercellular and anti-basement membrane zone antibodies. Blocking immunofluorescence and Western immunoblotting studies. (3069506)
1988
45
Complement fixation by Brazilian Pemphigus foliaceus autoantibodies. (2454770)
1988
46
Clinical, histologic, and immunopathologic comparison of pemphigus vulgaris and pemphigus foliaceus. (2978970)
1988
47
Pemphigus foliaceus coexisting with toxic, multinodular goiter. (3771047)
1986
48
Concurrent development of pemphigus foliaceus and psoriasis. (4018981)
1985
49
Brazilian pemphigus foliaceus. (7085163)
1982
50
On the association of some dermatoses with South American pemphigus foliaceus. (4371031)
1974

Variations for Pemphigus Foliaceus

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Expression for genes affiliated with Pemphigus Foliaceus

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pemphigus Foliaceus

Search GEO for disease gene expression data for Pemphigus Foliaceus.

Pathways for genes affiliated with Pemphigus Foliaceus

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50PathCards, 55Reactome, 38NCBI BioSystems Database, 30KEGG, 60Thomson Reuters
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Pathways related to Pemphigus Foliaceus according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.6DSG2, DSG1
29.5DSG1, HLA-DRB1
39.2EVPL, DST

Compounds for genes affiliated with Pemphigus Foliaceus

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45Novoseek, 51PharmGKB, 11DrugBank, 29IUPHAR, 24HMDB
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Compounds related to Pemphigus Foliaceus according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1ivig459.8CD79A, DSG1
2rituximab45 51 1111.7DSG1, CD79A
3neopterin459.6PLAUR, CD79A
4allergens459.5CD79A, HLA-DRB1
5suramin45 29 1111.5CD79A, PLAUR
6biotin45 24 1111.2CD79A, PLAUR
7paraffin458.9CD79A, PLAUR, DST
8arginine458.9CD79A, PLAUR, HLA-DRB1
9oligonucleotide458.4HLA-DRB1, DST, PLAUR

GO Terms for genes affiliated with Pemphigus Foliaceus

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16Gene Ontology
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Cellular components related to Pemphigus Foliaceus according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1lateral plasma membraneGO:0163289.6DSG2, DSG1
2cell-cell junctionGO:0059119.6DSG2, DSG1
3desmosomeGO:0300579.2EVPL, DSG2, DSG1
4plasma membraneGO:0058867.9HLA-DRB1, DSG1, DSG2, PLAUR, CD79A
5integral component of membraneGO:0160217.5CD79A, PLAUR, DSG2, DSG1, DST

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

idNameGO IDScoreTop Affiliating Genes
1cellular component disassembly involved in execution phase of apoptosisGO:0069219.6DSG2, DSG1
2maternal process involved in female pregnancyGO:0601359.5DSG2, DSG1
3response to progesteroneGO:0325709.3DSG2, DSG1

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

idNameGO IDScoreTop Affiliating Genes
1calcium ion bindingGO:0055098.8DSG2, DSG1, DST

Products for genes affiliated with Pemphigus Foliaceus

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Pemphigus Foliaceus

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4CDC
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
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet