MCID: PYD001
MIFTS: 48

Pyoderma Gangrenosum malady

Rare diseases, Skin diseases, Bone diseases categories

Summaries for Pyoderma Gangrenosum

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NIH Rare Diseases:41 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 based summary: Pyoderma Gangrenosum is related to colitis and ulcerative colitis, and has symptoms including skin rash, pulmonary infiltrates and myositis. 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 clofazimine and sulfapyridine pwdr and the compounds anakinra and methionine have been mentioned in the context of this disorder. Affiliated tissues include skin, neutrophil and breast, and related mouse phenotypes are skeleton and hematopoietic system.

Wikipedia:63 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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Sources:
9Disease Ontology, 41NIH Rare Diseases, 11DISEASES, 43Novoseek, 47Orphanet, 60UMLS, 33MeSH, 27ICD9CM, 55SNOMED-CT, 34MESH via Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet, 25ICD10
See all sources

Pyoderma Gangrenosum, Aliases & Descriptions:

Name: Pyoderma Gangrenosum 9 41 11 43 47 60


Classifications:

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


Characteristics (Orphanet epidemiological data):

47
pyoderma gangrenosum:
Age of onset: Adult; Age of death: normal life expectancy


External Ids:

Disease Ontology9 DOID:8553
MeSH33 D017511
ICD9CM27 686.01
Orphanet47 48104
SNOMED-CT55 74578003
MESH via Orphanet34 D017511
ICD10 via Orphanet26 L88
UMLS via Orphanet61 C0085652
ICD1025 L88

Related Diseases for Pyoderma Gangrenosum

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Diseases related to Pyoderma Gangrenosum via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 177)
idRelated DiseaseScoreTop Affiliating Genes
1colitis31.3IL1R1, NOD2
2ulcerative colitis31.1NOD2, IL1R1
3inflammatory bowel disease30.9NOD2, IL1R1, PSTPIP1
4psoriasis30.6NOD2, IL1R1
5rheumatoid arthritis30.3MEFV, IL1R1, NOD2
6systemic lupus erythematosus30.3NOD2, IL1R1
7cataract30.2MVK, IL1R1
8arthritis30.2MVK, NOD2, IL1R1, PSTPIP1, MEFV
9pyoderma11.5
10pyogenic arthritis, pyoderma gangrenosum and acne10.8
11acne10.8
12crohn's disease10.8
13myelodysplastic syndrome10.7
14hidradenitis10.7
15vasculitis10.6
16pyogenic sterile arthritis, pyoderma gangrenosum, and acne10.6
17breast reconstruction10.6
18hidradenitis suppurativa10.6
19subcorneal pustular dermatosis10.6
20hepatitis10.5
21leukemia10.5
22takayasu's arteritis10.5
23myeloma10.5
24leukocyte adhesion deficiency10.4
25collagenous colitis10.4
26dermatitis10.4
27lupus erythematosus10.4
28autoimmune hepatitis10.4
29behcet's disease10.4
30relapsing polychondritis10.4
31necrotizing fasciitis10.4
32sporotrichosis10.4
33fasciitis10.4
34osteomyelitis10.4
35scleritis10.4
36chronic recurrent multifocal osteomyelitis10.4
37erythema elevatum diutinum10.4
38pyoderma gangrenosum - acne - suppurative hidradenitis10.4
39multiple myeloma10.3
40burns10.3
41chronic myelomonocytic leukemia10.3
42myelofibrosis10.3
43neutropenia10.3
44hepatitis c10.3
45antiphospholipid syndrome10.3
46pemphigus10.3
47ecthyma10.3
48sapho syndrome10.3
49cicatricial ectropion10.3
50adenocarcinoma10.3

Graphical network of the top 20 diseases related to Pyoderma Gangrenosum:



Diseases related to pyoderma gangrenosum

Symptoms for Pyoderma Gangrenosum

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Symptoms:

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

HPO human phenotypes related to Pyoderma Gangrenosum:

id Description Frequency HPO Source Accession
1 skin rash hallmark (90%) HP:0000988
2 pulmonary infiltrates hallmark (90%) HP:0002113
3 myositis hallmark (90%) HP:0100614
4 skin ulcer hallmark (90%) HP:0200042
5 abnormal blistering of the skin typical (50%) HP:0008066

Drugs & Therapeutics for Pyoderma Gangrenosum

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Drug clinical trials:

Search ClinicalTrials for Pyoderma Gangrenosum

Search NIH Clinical Center for Pyoderma Gangrenosum

Inferred drug relations via UMLS60/NDF-RT39:

Genetic Tests for Pyoderma Gangrenosum

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Anatomical Context for Pyoderma Gangrenosum

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MalaCards organs/tissues related to Pyoderma Gangrenosum:

31
Skin, Neutrophil, Breast, Bone, Lung, T cells, Myeloid, Bone marrow, Monocytes, Liver, Prostate, Endothelial

Animal Models for Pyoderma Gangrenosum or affiliated genes

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MGI Mouse Phenotypes related to Pyoderma Gangrenosum:

35
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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Articles related to Pyoderma Gangrenosum:

(show top 50)    (show all 607)
idTitleAuthorsYear
1
Pyoderma gangrenosum secondary to azacitidine in myelodysplastic syndrome. (25765235)
2015
2
The Genetics of Pyoderma Gangrenosum and Implications for Treatment: A Systematic Review. (25350484)
2014
3
Recurrent pyoderma gangrenosum after cesarean delivery successfully treated with vacuum-assisted closure and split thickness skin graft: A case report. (25370183)
2014
4
Pyoderma gangrenosum with renal and splenic impairment - case report. (24346905)
2013
5
Penile pyoderma gangrenosum. (24339469)
2013
6
Letter to the editor: Temporary renal insufficiency associated with topical tacrolimus treatment of multilocal pyoderma gangrenosum. (24133568)
2013
7
Pyoderma gangrenosum in a newborn - case report. (24346911)
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
Penile pyoderma gangrenosum successfully treated with topical Imiquimod. (22021975)
2011
17
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
18
Hyperbaric oxygen therapy as an adjuvant treatment for pyoderma gangrenosum. (22281912)
2011
19
Pyoderma gangrenosum following trauma of the knee: a case of pathergy and review of orthopaedic cases. (20638661)
2011
20
Ciprofloxacin induced pyoderma gangrenosum. (23130244)
2011
21
Pyoderma gangrenosum of the breast after bilateral simple mastectomies for ductal carcinoma in situ. (21944335)
2011
22
Pyoderma gangrenosum associated with chronic idiopathic myelofibrosis after coronary artery bypass graft surgery. (19846444)
2010
23
Beclometasone inhaler used to treat pyoderma gangrenosum. (20500206)
2010
24
A case of pyoderma gangrenosum with ulcerative colitis treated with mesalazine. (21165212)
2010
25
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
26
Adalimumab treatment for peristomal pyoderma gangrenosum associated with Crohn's disease. (18942748)
2009
27
Early diagnosis and early corticosteroid administration improves healing of peristomal pyoderma gangrenosum in inflammatory bowel disease. (19279428)
2009
28
Successful use of adalimumab for treating fistulizing Crohn's disease with pyoderma gangrenosum: Two birds with one stone. (19437575)
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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Search GEO for disease gene expression data for Pyoderma Gangrenosum.

Pathways for genes affiliated with Pyoderma Gangrenosum

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Pathways related to Pyoderma Gangrenosum according to GeneCards Suite gene sharing:

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 pathways58
Immune response IL 1 signaling pathway58
9.1NOD2, IL1R1
3
Show member pathways
9.1IL1R1, NOD2
4
Show member pathways
IL-1 Signaling Pathway36
IL1-mediated signaling events36
9.1NOD2, IL1R1
5
Show member pathways
8.7NOD2, MEFV, PSTPIP1

Compounds for genes affiliated with Pyoderma Gangrenosum

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Sources:
43Novoseek, 12DrugBank, 49PharmGKB, 28IUPHAR
See all sources

Compounds related to Pyoderma Gangrenosum according to GeneCards Suite gene sharing:

idCompoundScoreTop Affiliating Genes
1anakinra43 1210.5IL1R1, MVK
2methionine439.4IL1R1, MEFV
3mevalonate439.3MVK, MEFV
4il 10439.3NOD2, IL1R1
5infliximab43 49 1211.2IL1R1, NOD2
6peptidoglycan43 2810.2NOD2, IL1R1
7endotoxin438.8NOD2, IL1R1, MEFV
8prednisolone43 28 1210.8NOD2, IL1R1
9threonine438.4MVK, IL1R1, NOD2
10serine438.2PSTPIP1, MVK, IL1R1, NOD2

GO Terms for genes affiliated with Pyoderma Gangrenosum

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Cellular components related to Pyoderma Gangrenosum according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cytosolGO:00058298.1NOD2, MVK, MEFV, PSTPIP1

Biological processes related to Pyoderma Gangrenosum according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1inflammatory responseGO:00069549.5PSTPIP1, MEFV
2negative regulation of inflammatory responseGO:00507289.2MEFV, MVK
3negative regulation of interleukin-12 productionGO:00326959.2NOD2, MEFV
4regulation of inflammatory responseGO:00507279.1IL1R1, NOD2
5nucleotide-binding domain, leucine rich repeat containing receptor signaling pathwayGO:00358728.8NOD2, MEFV, PSTPIP1
6innate immune responseGO:00450878.4PSTPIP1, MEFV, NOD2

Molecular functions related to Pyoderma Gangrenosum according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1actin bindingGO:00037799.5MEFV, PSTPIP1

Products for genes affiliated with Pyoderma Gangrenosum

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  • Antibodies
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  • Antibodies
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  • Kits and Assays

Sources for Pyoderma Gangrenosum

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet