MCID: PYD001
MIFTS: 46

Pyoderma Gangrenosum malady

Rare diseases, Skin diseases, Bone diseases categories

Aliases & Classifications for Pyoderma Gangrenosum

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Sources:
10Disease Ontology, 45NIH Rare Diseases, 47Novoseek, 12DISEASES, 51Orphanet, 65UMLS, 36MeSH, 59SNOMED-CT, 29ICD9CM, 28ICD10 via Orphanet, 37MESH via Orphanet, 66UMLS via Orphanet, 27ICD10
See all sources

Aliases & Descriptions for Pyoderma Gangrenosum:

Name: Pyoderma Gangrenosum 10 45 47 12 51 65 36


Classifications:



Characteristics (Orphanet epidemiological data):

51
pyoderma gangrenosum:
Age of onset: Adult; Age of death: normal life expectancy


External Ids:

Disease Ontology10 DOID:8553
MeSH36 D017511
ICD9CM29 686.01
Orphanet51 48104
SNOMED-CT59 74578003
ICD10 via Orphanet28 L88
MESH via Orphanet37 D017511
UMLS via Orphanet66 C0085652
ICD1027 L88

Summaries for Pyoderma Gangrenosum

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NIH Rare Diseases:45 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 dermatitis and hypersensitivity vasculitis, 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 Canonical NF-kappaB pathway and Immune response_MIF-mediated glucocorticoid regulation. The drugs clofazimine and sulfapyridine pwdr have been mentioned in the context of this disorder. Affiliated tissues include skin, neutrophil and breast, and related mouse phenotypes are hematopoietic system and immune system.

Wikipedia:68 Pyoderma gangrenosum is a condition that causes tissue to become necrotic, causing deep ulcers that... more...

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:

 51 (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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Drugs for Pyoderma Gangrenosum (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
Minocyclineapproved, investigationalPhase 316210118-90-85281021
Synonyms:
(2Z,4S,4aS,5aR,12aS)-2-[amino(hydroxy)methylidene]-4,7-bis(dimethylamino)-10,11,12a-trihydroxy-4a,5,5a,6-tetrahydro-4H-tetracene-1,3,12-trione
(4S,4AS,5AR,12AS)-4,7-BIS(DIMETHYLAMINO)-3,10,12,12A-TETRAHYDROXY-1,11-DIOXO-1,4,4A,5,5A,6,11,12A-OCTAHYDROTETRACENE-2-CARBOXAMIDE
(4S,4AS,5ar,12as)-4,7-bis(dimethylamino)-3,10,12,12a-tetrahydroxy-1,11-dioxo-1,4,4a,5,5a,6,11,12a-octahydrotetracene-2-carboxamide
10118-90-8
13614-98-7 (mono-hydrochloride)
4,7-Bis(dimethylamino)-1,4,4a,5,5a,6,11,12a-octahydro-3,10,12,12a-tetrahydroxy-1,11-dioxo-2-naphthacenecarboxamide
4708-96-7
7-Dimethylamino-6-demethyl-6-deoxytetracycline
AC1NQXWM
Arestin
BRN 3077644
Bio-0062
Borymycin
C07225
C23H27N3O7
CHEBI:50694
CHEBI:529981
CHEMBL1434
CHEMBL259172
CID5281021
CL 59806
CRL-1605 & Minocycline
D05045
Dynacin
HMS2090D03
HSDB 3130
LMPK07000002
 
LS-93850
Lactoferrin B & Minocycline
Lactoferrin H & Minocycline
MINO
MIY
Minociclina
Minociclina [INN-Spanish]
Minociclinum
Minocin
Minocin (Hydrochloride)
Minocline
Minocyclin
Minocycline
Minocycline (USAN/INN)
Minocycline Hydrochloride
Minocycline Monohydrochloride
Minocycline [USAN:BAN:INN]
Minocyclinum
Minocyclinum [INN-Latin]
NCGC00178854-01
NSC 141993
NSC141993
Solodyn
UNII-FYY3R43WGO
Vectrin (Hydrochloride)
Ximino
minociclinum
minocycline
nchembio.559-comp1
2
Tacrolimusapproved, investigationalPhase 2992104987-11-3445643, 439492, 445647, 439492, 445647
Synonyms:
(-)-FK 506
(3S,4R,5S,8R,9E,12S,14S,15R,16S,18R,19R,26aS)-5,19-dihydroxy-3-{(1E)-1-[(1R,3R,4R)-4-hydroxy-3-methoxycyclohexyl]prop-1-en-2-yl}-14,16-dimethoxy-4,10,12,18-tetramethyl-8-(prop-2-en-1-yl)-5,6,8,11,12,13,14,15,16,17,18,19,24,25,26,26a-hexadecahydro-3H-15,19-epoxypyrido[2,1-c][1,4]oxazacyclotricosine-1,7,20,21(4H,23H)-tetrone
104987-11-3
109581-93-3 (Hydrate)
15,19-epoxy-3H-pyrido[2,1-c][1,4]oxaazacyclotricosine-1,7,20,21(23H)-tetrone,
3S-[3R*[E(1S*,3S*,4S*)],4S*,5R*,8S*,9E,12R*,14R*,15S*,16R*,18S*,19S*,26aR*]]-5,6,8,11,12,13,14,15,16,17,18,19,24,25,26,26a-hexadecahydro-5, 19-dihydroxy-3-[2-(4-hydroxy-3-methoxycyclohexyl)-1-methylethenyl]-14,16-dimethoxy-4,10,12,18-tetramethyl-8-(2-propenyl)-15,19-epoxy-3H-pyrido[2,1-c] [1,4] oxaazacyclotricosine-1,7,20,21(4H,23H)-tetrone
8-DEETHYL-8-[BUT-3-ENYL]-ASCOMYCIN
8-DEETHYL-8-[but-3-enyl]-ascomycin
AC-1182
AC1L1K7H
AC1L97GB
AC1L9IBU
AKOS005145901
Advagraf
Ambap104987-11-3
Ambap5429
BCBcMAP01_000194
BRD-K35452788-001-02-1
BSPBio-001279
BSPBio_001279
Bio-0921
Bio2_000470
Bio2_000950
C01375
C44H69NO10
CCRIS 7124
CHEBI:100924
CHEBI:61049
CHEMBL1200738
CID11158639
CID11556866
CID439492
CID445643
CID5372
CID5472317
CID6426916
CID6436007
CID6473866
CID6536850
CID6610362
CID6912836
CID9832283
CID9853905
CID9918805
CID9940643
CID9963169
CPD-10016
CPD000466356
D08556
DB00864
DivK1c_001040
FK 506
FK-506
FK5
FK506
FR 900506
FR-900506
FR900506
 
FT-0082660
Fk-506
Fujimycin
Graceptor
HMS1362O21
HMS1792O21
HMS1990O21
HMS2051C18
HMS2093M19
HMS503O21
Hecoria
IDI1_001040
IDI1_002225
K506
KBio1_001040
KBio2_000619
KBio2_003187
KBio2_005755
KBio3_001097
KBio3_001098
KBioGR_000619
KBioSS_000619
L 679934
L-679934
LCP-Tacro
LMPK04000003
LS-64247
MLS000759471
MLS001424054
Modigraf
MolPort-003-666-518
NCGC00163470-01
NCGC00163470-02
NCGC00163470-03
NCGC00163470-04
NCGC00179232-01
NChemBio.2007.16-comp1
NINDS_001040
NSC717865
Prograf
Prograf (TN)
Protopic
Protopy
S5003_Selleck
SAM001246677
SMR000466356
Tacarolimus
Tacrolimus
Tacrolimus (INN)
Tacrolimus (Prograf?)
Tacrolimus (anhydrous)
Tacrolimus Hydrate
Tacrolimus anhydrous
Tacrolimus hydrate
Tsukubaenolide
UNII-Y5L2157C4J
nchembio.2007.23-comp2
tacrolimus
tacrolimus hydrate
3
Infliximabapproved327170277-31-3
Synonyms:
Ig gamma-1 chain C region
 
Inflectra
Remicade
Remsima

Interventional clinical trials:

(show all 13)
idNameStatusNCT IDPhase
1An Efficacy and Safety Study of Gevokizumab in Treating Active Ulcers of Pyoderma GangrenosumRecruitingNCT02326740Phase 3
2An Efficacy and Safety Study of Gevokizumab in Treating Active Ulcers of Pyoderma GangrenosumRecruitingNCT02315417Phase 3
3Minocycline Therapy for Lung Scarring in Patients With Idiopathic Pulmonary Fibrosis - a Pilot StudyActive, not recruitingNCT00203697Phase 3
4A 2-Year, Open-Label, Safety Extension Study of Gevokizumab in Subjects With Pyoderma GangrenosumEnrolling by invitationNCT02318914Phase 3
5Proof of Concept Study of Gevokizumab in the Treatment of Pyoderma GangrenosumCompletedNCT01882504Phase 2
6Topical Tacrolimus 0.1% Ointment For Treatment Of Cutaneous Crohn's DiseaseCompletedNCT01233570Phase 2
7RA-18C3 Therapy for Subjects With Pyoderma GangrenosumRecruitingNCT01965613Phase 2
8Canakinumab for Pyoderma GangrenosumRecruitingNCT01302795Phase 2
9Safety and Efficacy Study of Humira in Treatment of Pyoderma GangrenosumWithdrawnNCT00730717Phase 2
10Study to Determine the Safety and Efficacy of Adalimumab in the Treatment of Pyoderma GangrenosumWithdrawnNCT00690846Phase 2
11Open Label Study for Adults With Pyoderma Gangrenosum and Inflammatory Bowel DiseaseCompletedNCT00791557
12Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin DiseasesRecruitingNCT01952275
13Schnitzler Syndrome: Clinical Study, Physiopathological and Search for Genetic FactorsRecruitingNCT00933296

Search NIH Clinical Center for Pyoderma Gangrenosum

Inferred drug relations via UMLS65/NDF-RT43:


Cochrane evidence based reviews: Pyoderma Gangrenosum

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:

33
Skin, Neutrophil, Breast, Bone, Lung, T cells, Myeloid

Animal Models for Pyoderma Gangrenosum or affiliated genes

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MGI Mouse Phenotypes related to Pyoderma Gangrenosum:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053977.7CXCL8, IL1R1, MEFV, NLRP3, NOD2, PSTPIP1
2MP:00053876.6CXCL8, IL1R1, MEFV, MMP10, NLRP3, NOD2

Publications for Pyoderma Gangrenosum

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Articles related to Pyoderma Gangrenosum:

(show top 50)    (show all 624)
idTitleAuthorsYear
1
Granulomatous pyoderma gangrenosum of the genitalia in the absence of Crohn's disease. (26392658)
2015
2
Pyoderma gangrenosum secondary to azacitidine in myelodysplastic syndrome. (25765235)
2015
3
The Genetics of Pyoderma Gangrenosum and Implications for Treatment: A Systematic Review. (25350484)
2014
4
Recurrent pyoderma gangrenosum after cesarean delivery successfully treated with vacuum-assisted closure and split thickness skin graft: A case report. (25370183)
2014
5
Pyoderma gangrenosum with renal and splenic impairment - case report. (24346905)
2013
6
Penile pyoderma gangrenosum. (24339469)
2013
7
Letter to the editor: Temporary renal insufficiency associated with topical tacrolimus treatment of multilocal pyoderma gangrenosum. (24133568)
2013
8
Pyoderma gangrenosum in a newborn - case report. (24346911)
2013
9
Dermacase. Can you identify this condition? Pyoderma gangrenosum. (23585606)
2013
10
Auricular pyoderma gangrenosum associated with Crohn's disease. (22585655)
2012
11
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
12
Refractory pyoderma gangrenosum associated with ulcerative colitis successfully treated with infliximab. (22301049)
2012
13
Pyoderma gangrenosum in breast reconstruction. (22747349)
2012
14
Incidence, mortality, and disease associations of pyoderma gangrenosum in the United Kingdom: a retrospective cohort study. (22534879)
2012
15
Pyoderma gangrenosum in a patient with primary cutaneous peripheral T-cell lymphoma, not otherwise specified. (23228951)
2012
16
Retinal vasculitis associated with pyoderma gangrenosum: a case report. (22669709)
2012
17
Pyoderma gangrenosum-like ulcer caused by Helicobacter cinaedi in a patient with x-linked agammaglobulinaemia. (22439627)
2012
18
Penile pyoderma gangrenosum successfully treated with topical Imiquimod. (22021975)
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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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:

(show all 18)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.8NOD2, TNF
29.6CXCL8, TNF
39.6CXCL8, TNF
49.2CXCL8, NLRP3, TNF
59.2CXCL8, NLRP3, TNF
69.2IFNA2, NOD2, TNF
79.1CXCL8, IL1R1, TNF
89.1CXCL8, IL1R1, TNF
9
Show member pathways
9.1CXCL8, IL1R1, TNF
10
Show member pathways
9.1CXCL8, IL1R1, TNF
119.1CXCL8, IL1R1, TNF
12
Show member pathways
9.0CXCL8, IFNA2, TNF
139.0CXCL8, IFNA2, TNF
14
Show member pathways
9.0CXCL8, IFNA2, TNF
15
Show member pathways
8.6CXCL8, IFNA2, NLRP3, TNF
16
Show member pathways
8.2CXCL8, MEFV, NLRP3, NOD2, PSTPIP1, TNF
17
Show member pathways
7.8CXCL8, IFNA2, IL1R1, NLRP3, NOD2, TNF
18
Show member pathways
7.6IFNA2, IL1R1, MEFV, NLRP3, NOD2, PSTPIP1

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
1extracellular spaceGO:00056157.6CXCL8, IFNA2, IL1R1, MMP10, TNF

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

(show all 20)
idNameGO IDScoreTop Affiliating Genes
1detection of biotic stimulusGO:000959510.4NLRP3, NOD2
2positive regulation of humoral immune response mediated by circulating immunoglobulinGO:000292510.4NOD2, TNF
3positive regulation of NIK/NF-kappaB signalingGO:190122410.4NOD2, TNF
4negative regulation of interleukin-12 productionGO:003269510.3MEFV, NOD2
5positive regulation of interleukin-1 beta secretionGO:005071810.3NLRP3, NOD2
6positive regulation of type 2 immune responseGO:000283010.3NLRP3, NOD2
7positive regulation of interleukin-8 productionGO:003275710.3NOD2, TNF
8negative regulation of growth of symbiont in hostGO:004413010.2NOD2, TNF
9positive regulation of cysteine-type endopeptidase activity involved in apoptotic processGO:004328010.2NLRP3, TNF
10positive regulation of phagocytosisGO:005076610.1NOD2, TNF
11embryonic digestive tract developmentGO:004856610.0CXCL8, TNF
12positive regulation of NF-kappaB transcription factor activityGO:00510929.9NLRP3, NOD2, TNF
13negative regulation of inflammatory responseGO:00507289.7MEFV, MVK, NLRP3
14response to exogenous dsRNAGO:00433309.7IFNA2, NOD2
15nucleotide-binding domain, leucine rich repeat containing receptor signaling pathwayGO:00358729.6MEFV, NLRP3, NOD2, PSTPIP1
16cellular response to lipopolysaccharideGO:00712229.6CXCL8, NLRP3, TNF
17defense responseGO:00069529.1IFNA2, NLRP3, NOD2, TNF
18regulation of inflammatory responseGO:00507279.0IL1R1, NLRP3, NOD2
19innate immune responseGO:00450878.5IFNA2, MEFV, NLRP3, NOD2, PSTPIP1
20inflammatory responseGO:00069548.2CXCL8, IFNA2, MEFV, NLRP3, PSTPIP1, TNF

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

idNameGO IDScoreTop Affiliating Genes
1peptidoglycan bindingGO:00428349.8NLRP3, NOD2

Sources for Pyoderma Gangrenosum

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet