MCID: PYD001
MIFTS: 56

Pyoderma Gangrenosum malady

Rare diseases, Skin diseases, Bone diseases categories
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Summaries for Pyoderma Gangrenosum

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NIH Rare Diseases:42 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 chronic skin infection/ulcerations/ulcers/cancrum, cutaneous rash and lung/pulmonary infiltrates. 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 neutrophil, skin and breast, and related mouse phenotypes are skeleton and hematopoietic system.

Wikipedia:65 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:
8Disease Ontology, 42NIH Rare Diseases, 10DISEASES, 44Novoseek, 48Orphanet, 62UMLS, 57SNOMED-CT, 27ICD9CM, 34MeSH, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet, 25ICD10
See all sources

Pyoderma Gangrenosum, Aliases & Descriptions:

Name: Pyoderma Gangrenosum 8 42 10 44 48 62


Classifications:

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


Characteristics (Orphanet epidemiological data):

48
pyoderma gangrenosum:
Age of onset: Adulthood; Age of death: Normal


External Ids:

Disease Ontology8 DOID:8553
ICD9CM27 686.01
MeSH34 D017511
MESH via Orphanet35 D017511
SNOMED-CT57 74578003
ICD10 via Orphanet26 L88
UMLS via Orphanet63 C0085652
ICD1025 L88

Related Diseases for Pyoderma Gangrenosum

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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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Symptoms:

48 (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:

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Search NIH Clinical Center for Pyoderma Gangrenosum

Inferred drug relations via UMLS62/NDF-RT40:

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:

32
Neutrophil, Skin, 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:

36
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 579)
idTitleAuthorsYear
1
The Genetics of Pyoderma Gangrenosum and Implications for Treatment: A Systematic Review. (25350484)
2014
2
Recurrent pyoderma gangrenosum after cesarean delivery successfully treated with vacuum-assisted closure and split thickness skin graft: A case report. (25370183)
2014
3
Pyoderma gangrenosum with renal and splenic impairment - case report. (24346905)
2013
4
Penile pyoderma gangrenosum. (24339469)
2013
5
Letter to the editor: Temporary renal insufficiency associated with topical tacrolimus treatment of multilocal pyoderma gangrenosum. (24133568)
2013
6
Pyoderma gangrenosum in a newborn - case report. (24346911)
2013
7
Dermacase. Can you identify this condition? Pyoderma gangrenosum. (23585606)
2013
8
Genital pyoderma gangrenosum: report of two cases and published work review of Japanese cases. (24033392)
2013
9
Auricular pyoderma gangrenosum associated with Crohn's disease. (22585655)
2012
10
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
11
Refractory pyoderma gangrenosum associated with ulcerative colitis successfully treated with infliximab. (22301049)
2012
12
Pyoderma gangrenosum in breast reconstruction. (22747349)
2012
13
Incidence, mortality, and disease associations of pyoderma gangrenosum in the United Kingdom: a retrospective cohort study. (22534879)
2012
14
Pyoderma gangrenosum in a patient with primary cutaneous peripheral T-cell lymphoma, not otherwise specified. (23228951)
2012
15
Retinal vasculitis associated with pyoderma gangrenosum: a case report. (22669709)
2012
16
Pyoderma gangrenosum-like ulcer caused by Helicobacter cinaedi in a patient with x-linked agammaglobulinaemia. (22439627)
2012
17
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
18
Penile pyoderma gangrenosum successfully treated with topical Imiquimod. (22021975)
2011
19
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
20
Hyperbaric oxygen therapy as an adjuvant treatment for pyoderma gangrenosum. (22281912)
2011
21
Pyoderma gangrenosum following trauma of the knee: a case of pathergy and review of orthopaedic cases. (20638661)
2011
22
Ciprofloxacin induced pyoderma gangrenosum. (23130244)
2011
23
Pyoderma gangrenosum of the breast after bilateral simple mastectomies for ductal carcinoma in situ. (21944335)
2011
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
Successful use of adalimumab for treating fistulizing Crohn's disease with pyoderma gangrenosum: Two birds with one stone. (19437575)
2009
30
Seat belt pyoderma gangrenosum: minor pressure as a causative factor. (18837131)
2008
31
Pyoderma gangrenosum of the scalp following hair highlights in a postpartum patient. (18086612)
2008
32
An open-label pilot study of alefacept for the treatment of pyoderma gangrenosum. (18384543)
2008
33
A novel therapy for the treatment of pyoderma gangrenosum. (18935734)
2008
34
Pyoderma gangrenosum in the head and neck. (17224531)
2007
35
Negative pressure dressing in the management of pyoderma gangrenosum ulcer. (17938337)
2007
36
Pulmonary manifestations of Pyoderma gangrenosum. (17555913)
2007
37
Pyoderma gangrenosum in an infant. (16918628)
2006
38
The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. (16415387)
2006
39
A case of pyoderma gangrenosum responding to high-dose intravenous immunoglobulin therapy. (16863619)
2006
40
An exuberant inflammatory response to E coli: implications for the pathogenesis of ulcerative colitis and pyoderma gangrenosum. (17047113)
2006
41
Bilateral pyoderma gangrenosum complicating minimal access cranial suspension face lift. (19338926)
2006
42
Use of etanercept in treatment of pyoderma gangrenosum in a patient with autoimmune hepatitis. (16428159)
2005
43
Small joint monoarthritis and pyoderma gangrenosum in newly diagnosed ulcerative colitis: an unusual presentation. (17043551)
2004
44
Pyoderma gangrenosum complicated by ecthyma gangrenosum. (15149521)
2004
45
Treatment of pyoderma gangrenosum with etanercept. (15303791)
2004
46
A case of SAPHO syndrome with pyoderma gangrenosum and inflammatory bowel disease masquerading as BehAset's disease. (12918720)
2003
47
A new autosomal dominant disorder of pyogenic sterile arthritis, pyoderma gangrenosum, and acne: PAPA syndrome. (9212761)
1997
48
Pyoderma gangrenosum associated with cystic acne and hidradenitis suppurativa controlled by adding minocycline and sulfasalazine to the treatment regimen. (8726710)
1996
49
Klippel-Trenaunay-Weber syndrome mimicking pyoderma gangrenosum. (8537163)
1995
50
Pyoderma gangrenosum in association with erythema elevatum diutinum: report of two cases. (1572226)
1992

Variations for Pyoderma Gangrenosum

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Expression for genes affiliated with Pyoderma Gangrenosum

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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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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 pathways60
Immune response IL 1 signaling pathway60
9.1NOD2, IL1R1
3
Show member pathways
9.1IL1R1, NOD2
4
Show member pathways
IL-1 Signaling Pathway37
IL1-mediated signaling events37
9.1NOD2, IL1R1
5
Show member pathways
8.7NOD2, MEFV, PSTPIP1

Compounds for genes affiliated with Pyoderma Gangrenosum

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Sources:
44Novoseek, 11DrugBank, 50PharmGKB, 28IUPHAR
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Compounds related to Pyoderma Gangrenosum according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1anakinra44 1110.5IL1R1, MVK
2methionine449.4IL1R1, MEFV
3mevalonate449.3MVK, MEFV
4il 10449.3NOD2, IL1R1
5infliximab44 50 1111.2IL1R1, NOD2
6peptidoglycan44 2810.2NOD2, IL1R1
7endotoxin448.8NOD2, IL1R1, MEFV
8prednisolone44 28 1110.8NOD2, IL1R1
9threonine448.4MVK, IL1R1, NOD2
10serine448.2PSTPIP1, MVK, IL1R1, NOD2

GO Terms for genes affiliated with Pyoderma Gangrenosum

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Cellular components related to Pyoderma Gangrenosum according to GeneCards/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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Sources for Pyoderma Gangrenosum

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet