MCID: PYD001
MIFTS: 55

Pyoderma Gangrenosum malady

Rare diseases, Skin diseases, Bone diseases categories

Summaries for Pyoderma Gangrenosum

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Sources:
44NIH Rare Diseases, 66Wikipedia, 34MalaCards
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NIH Rare Diseases:44 Pyoderma gangrenosum is a rare, destructive inflammatory skin disease of which a painful nodule or pustule breaks down to form a progressively enlarging ulcer. lesions may occur either in the absence of any apparent underlying disorder or in association with other diseases, such as ulcerative colitis, crohn's disease, polyarthritis (an inflammation of several joints together), gammopathy, and other conditions . pyoderma gangrenosum belongs to a group of skin diseases in which a common cellular denominator is the neutrophil. neutrophils are a type of white blood cell or leukocyte which form an early line of defense against bacterial infections.  each year in the united states, pyoderma gangrenosum occurs in about 1 person per 100.000 people. last updated: 4/26/2011

MalaCards: Pyoderma Gangrenosum is related to ulcerative colitis and inflammatory bowel disease, and has symptoms including lung/pulmonary infiltrates, myositis and vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment. An important gene associated with Pyoderma Gangrenosum is PSTPIP1 (proline-serine-threonine phosphatase interacting protein 1), and among its related pathways are Interferon Signaling and NOD-like Receptor Signaling Pathways. The drugs sulfapyridine pwdr and clofazimine and the compounds anakinra and methionine have been mentioned in the context of this disorder. Affiliated tissues include skin, breast and lung, and related mouse phenotypes are skeleton and hematopoietic system.

Wikipedia:66 Pyoderma gangrenosum is a condition that causes tissue to become necrotic, causing deep ulcers that... more...

Aliases & Classifications for Pyoderma Gangrenosum

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9Disease Ontology, 44NIH Rare Diseases, 11DISEASES, 46Novoseek, 50Orphanet, 63UMLS, 36MeSH, 59SNOMED-CT, 28ICD9CM, 27ICD10 via Orphanet, 60SNOMED-CT via Orphanet, 64UMLS via Orphanet, 37MESH via Orphanet, 26ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases, Bone diseases


Characteristics (Orphanet epidemiological data):

50
pyoderma gangrenosum:
Age of onset: Adulthood; Age of death: Normal


Aliases & Descriptions:

pyoderma gangrenosum 9 44 11 46 50 63


External Ids:

Disease Ontology9 DOID:8553
MeSH36 D017511
ICD9CM28 686.01
ICD10 via Orphanet27 L88
SNOMED-CT59 74578003
SNOMED-CT via Orphanet60 74578003
UMLS via Orphanet64 C0085652
MESH via Orphanet37 D017511
ICD1026 L88

Related Diseases for Pyoderma Gangrenosum

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18GeneCards, 19GeneDecks
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Graphical network of the top 20 diseases related to Pyoderma Gangrenosum:



Diseases related to pyoderma gangrenosum

Symptoms for Pyoderma Gangrenosum

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50Orphanet
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Symptoms:

50 (show all 8)
  • lung/pulmonary infiltrates
  • myositis
  • vesicles/bullous/exsudative lesions/bullous/cutaneous/mucosal detachment
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • cutaneous rash
  • chronic skin infection/ulcerations/ulcers/cancrum
  • subcutaneous nodules/lipomas/tumefaction/swelling
  • dermal/subcutaneous infiltration/induration

Drugs & Therapeutics for Pyoderma Gangrenosum

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Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
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Approved drugs:

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Drug clinical trials:

Search ClinicalTrials for Pyoderma Gangrenosum

Search NIH Clinical Center for Pyoderma Gangrenosum

Search CenterWatch for Pyoderma Gangrenosum

Inferred drug relations via UMLS63/NDF-RT42:

Genetic Tests for Pyoderma Gangrenosum

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Anatomical Context for Pyoderma Gangrenosum

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34MalaCards
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MalaCards organs/tissues related to Pyoderma Gangrenosum:

34
Skin, Breast, Lung, Bone, T cells, Myeloid, Monocytes, Bone marrow, Endothelial, Prostate, Liver

Animal Models for Pyoderma Gangrenosum or affiliated genes

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38MGI
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MGI Mouse Phenotypes related to Pyoderma Gangrenosum:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053908.6NOD2, IL1R1, MEFV
2MP:00053978.1NOD2, IL1R1, MEFV, PSTPIP1
3MP:00053877.9PSTPIP1, MEFV, IL1R1, NOD2

Publications for Pyoderma Gangrenosum

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53PubMed
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Articles related to Pyoderma Gangrenosum:

(show top 50)    (show all 558)
idTitleAuthorsYear
1
Pyoderma gangrenosum with renal and splenic impairment - case report. (24346905)
2013
2
Penile pyoderma gangrenosum. (24339469)
2013
3
Letter to the editor: Temporary renal insufficiency associated with topical tacrolimus treatment of multilocal pyoderma gangrenosum. (24133568)
2013
4
Pyoderma gangrenosum in a newborn - case report. (24346911)
2013
5
Dermacase. Can you identify this condition? Pyoderma gangrenosum. (23585606)
2013
6
Genital pyoderma gangrenosum: report of two cases and published work review of Japanese cases. (24033392)
2013
7
Pyoderma gangrenosum following foot and ankle surgery: a case report. (23637240)
2013
8
Auricular pyoderma gangrenosum associated with Crohn's disease. (22585655)
2012
9
Pyogenic arthritis, pyoderma gangrenosum, and acne syndrome (PAPA syndrome) with E250K mutation in CD2BP1 gene treated with the tumor necrosis factor inhibitor adalimumab. (22513199)
2012
10
Refractory pyoderma gangrenosum associated with ulcerative colitis successfully treated with infliximab. (22301049)
2012
11
Pyoderma gangrenosum in breast reconstruction. (22747349)
2012
12
Incidence, mortality, and disease associations of pyoderma gangrenosum in the United Kingdom: a retrospective cohort study. (22534879)
2012
13
Pyoderma gangrenosum in a patient with primary cutaneous peripheral T-cell lymphoma, not otherwise specified. (23228951)
2012
14
Retinal vasculitis associated with pyoderma gangrenosum: a case report. (22669709)
2012
15
Pyoderma gangrenosum-like ulcer caused by Helicobacter cinaedi in a patient with x-linked agammaglobulinaemia. (22439627)
2012
16
A mechanically powered negative pressure device used in conjunction with a bioengineered cell-based product for the treatment of pyoderma gangrenosum: a case report i>?. (22933700)
2012
17
Penile pyoderma gangrenosum successfully treated with topical Imiquimod. (22021975)
2011
18
Staged correction of an equinovarus deformity due to pyoderma gangrenosum using a Taylor spatial frame and tibiotalar calcaneal fusion with an intramedullary device. (21863298)
2011
19
Hyperbaric oxygen therapy as an adjuvant treatment for pyoderma gangrenosum. (22281912)
2011
20
Pyoderma gangrenosum following trauma of the knee: a case of pathergy and review of orthopaedic cases. (20638661)
2011
21
Ciprofloxacin induced pyoderma gangrenosum. (23130244)
2011
22
Pyoderma gangrenosum of the breast after bilateral simple mastectomies for ductal carcinoma in situ. (21944335)
2011
23
Pyoderma gangrenosum associated with chronic idiopathic myelofibrosis after coronary artery bypass graft surgery. (19846444)
2010
24
Beclometasone inhaler used to treat pyoderma gangrenosum. (20500206)
2010
25
A case of pyoderma gangrenosum with ulcerative colitis treated with mesalazine. (21165212)
2010
26
Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra. (19673875)
2009
27
Adalimumab treatment for peristomal pyoderma gangrenosum associated with Crohn's disease. (18942748)
2009
28
Early diagnosis and early corticosteroid administration improves healing of peristomal pyoderma gangrenosum in inflammatory bowel disease. (19279428)
2009
29
Seat belt pyoderma gangrenosum: minor pressure as a causative factor. (18837131)
2008
30
Pyoderma gangrenosum of the scalp following hair highlights in a postpartum patient. (18086612)
2008
31
An open-label pilot study of alefacept for the treatment of pyoderma gangrenosum. (18384543)
2008
32
A novel therapy for the treatment of pyoderma gangrenosum. (18935734)
2008
33
Pyoderma gangrenosum in the head and neck. (17224531)
2007
34
Negative pressure dressing in the management of pyoderma gangrenosum ulcer. (17938337)
2007
35
Pulmonary manifestations of Pyoderma gangrenosum. (17555913)
2007
36
Pyoderma gangrenosum in an infant. (16918628)
2006
37
The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. (16415387)
2006
38
A case of pyoderma gangrenosum responding to high-dose intravenous immunoglobulin therapy. (16863619)
2006
39
An exuberant inflammatory response to E coli: implications for the pathogenesis of ulcerative colitis and pyoderma gangrenosum. (17047113)
2006
40
Bilateral pyoderma gangrenosum complicating minimal access cranial suspension face lift. (19338926)
2006
41
Use of etanercept in treatment of pyoderma gangrenosum in a patient with autoimmune hepatitis. (16428159)
2005
42
Small joint monoarthritis and pyoderma gangrenosum in newly diagnosed ulcerative colitis: an unusual presentation. (17043551)
2004
43
Pyoderma gangrenosum complicated by ecthyma gangrenosum. (15149521)
2004
44
Treatment of pyoderma gangrenosum with etanercept. (15303791)
2004
45
A case of SAPHO syndrome with pyoderma gangrenosum and inflammatory bowel disease masquerading as BehAset's disease. (12918720)
2003
46
A new autosomal dominant disorder of pyogenic sterile arthritis, pyoderma gangrenosum, and acne: PAPA syndrome. (9212761)
1997
47
Pyoderma gangrenosum associated with cystic acne and hidradenitis suppurativa controlled by adding minocycline and sulfasalazine to the treatment regimen. (8726710)
1996
48
Klippel-Trenaunay-Weber syndrome mimicking pyoderma gangrenosum. (8537163)
1995
49
Pyoderma gangrenosum in association with erythema elevatum diutinum: report of two cases. (1572226)
1992
50
Pyoderma gangrenosum followed by subcorneal pustular dermatosis in a patient with IgA paraproteinemia. (1999540)
1991

Variations for Pyoderma Gangrenosum

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Expression for genes affiliated with Pyoderma Gangrenosum

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2BioGPS, 16Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Pyoderma Gangrenosum

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Pathways for genes affiliated with Pyoderma Gangrenosum

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51PathCards, 56Reactome, 55R&D Systems, 61Thomson Reuters, 39NCBI BioSystems Database, 31KEGG
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Pathways related to Pyoderma Gangrenosum according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.1NOD2, IL1R1
2
Show member pathways
Immune response Role of TLRs 3 and 4 in cell antiviral response TICAM1 specific signaling pathways61
Immune response IL 1 signaling pathway61
9.1NOD2, IL1R1
3
Show member pathways
9.1IL1R1, NOD2
4
Show member pathways
IL-1 Signaling Pathway39
IL1-mediated signaling events39
9.1NOD2, IL1R1
5
Show member pathways
8.7NOD2, MEFV, PSTPIP1

Compounds for genes affiliated with Pyoderma Gangrenosum

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46Novoseek, 12DrugBank, 52PharmGKB, 30IUPHAR
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Compounds related to Pyoderma Gangrenosum according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1anakinra46 1210.5IL1R1, MVK
2methionine469.4IL1R1, MEFV
3mevalonate469.3MVK, MEFV
4il 10469.3NOD2, IL1R1
5infliximab46 52 1211.2IL1R1, NOD2
6peptidoglycan46 3010.2NOD2, IL1R1
7endotoxin468.8NOD2, IL1R1, MEFV
8prednisolone46 30 1210.8NOD2, IL1R1
9threonine468.4MVK, IL1R1, NOD2
10serine468.2PSTPIP1, MVK, IL1R1, NOD2

GO Terms for genes affiliated with Pyoderma Gangrenosum

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17Gene Ontology
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Cellular components related to Pyoderma Gangrenosum according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cytosolGO:0058298.1NOD2, MVK, MEFV, PSTPIP1

Biological processes related to Pyoderma Gangrenosum according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1inflammatory responseGO:0069549.5MEFV, PSTPIP1
2negative regulation of inflammatory responseGO:0507289.2MEFV, MVK
3negative regulation of interleukin-12 productionGO:0326959.2NOD2, MEFV
4regulation of inflammatory responseGO:0507279.1NOD2, IL1R1
5nucleotide-binding domain, leucine rich repeat containing receptor signaling pathwayGO:0358728.8NOD2, MEFV, PSTPIP1
6innate immune responseGO:0450878.4NOD2, MEFV, PSTPIP1

Molecular functions related to Pyoderma Gangrenosum according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1actin bindingGO:0037799.5MEFV, PSTPIP1

Products for genes affiliated with Pyoderma Gangrenosum

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Pyoderma Gangrenosum

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4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet