MCID: PYD001
MIFTS: 51

Pyoderma Gangrenosum malady

Categories: Rare diseases, Skin diseases, Bone diseases

Aliases & Classifications for Pyoderma Gangrenosum

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

Aliases & Descriptions for Pyoderma Gangrenosum:

Name: Pyoderma Gangrenosum 10 45 47 12 51 36 65

Characteristics:

Orphanet epidemiological data:

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

Classifications:



External Ids:

Disease Ontology10 DOID:8553
ICD1027 L88
ICD9CM29 686.01
MeSH36 D017511
SNOMED-CT59 74578003
Orphanet51 48104
ICD10 via Orphanet28 L88
MESH via Orphanet37 D017511
UMLS via Orphanet66 C0085652
UMLS65 C0085652

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 hidradenitis and ileocolitis, 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 Transcription_NF-kB signaling pathway and Monoamine Transport. The drugs sulfapyridine pwdr and clofazimine 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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Diseases related to Pyoderma Gangrenosum via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50)    (show all 232)
idRelated DiseaseScoreTop Affiliating Genes
1hidradenitis31.1NOD2, TNF
2ileocolitis30.8NOD2, TNF
3cryoglobulinemia30.1NOD2, TNF
4pyogenic arthritis, pyoderma gangrenosum and acne12.5
5pyogenic sterile arthritis, pyoderma gangrenosum, and acne12.5
6pyoderma gangrenosum-acne-suppurative hidradenitis syndrome12.2
7pyoderma11.6
8polydactyly, postaxial, types a1 and b11.2
9crohn's disease10.9
10colitis10.6
11ulcerative colitis10.6
12occipital lobe neoplasm10.6PSTPIP1, TNF
13pyogenic granuloma10.5IL1R1, PSTPIP1
14precursor b lymphoblastic lymphoma/leukemia10.5IL1R1, TNF
15turner syndrome10.5IL1R1, TNF
16acne10.5
17arthritis10.5
18kernicterus due to isoimmunization10.5IFNA2, TNF
19pustulosis of palm and sole10.4NOD2, TNF
20gastroduodenal crohn's disease10.4NOD2, TNF
21capillary lymphangioma10.4NOD2, TNF
22trichostrongyloidiasis10.4IFNA2, TNF
23cryptomicrotia brachydactyly syndrome10.4IL1R1, NLRP3
24spondylocamptodactyly10.3NOD2, TNF
25chromosome 9p deletion syndrome10.3NOD2, TNF
26myelodysplastic syndrome10.3
27lymphoma10.3
28peripheral retinal degeneration10.3NLRP3, TNF
29intracranial embolism10.3IL1R1, NLRP3
30collagen vi related muscular dystrophy10.3IL1R1, NLRP3
31familial colorectal cancer10.3IL1R1, NLRP3
32trench fever10.3NOD2, TNF
33cauda equina syndrome10.3IL1R1, NOD2
34wells-jankovic syndrome10.3IL1R1, NLRP3
35rhinoscleroma10.3MEFV, TNF
36acute leukemia of ambiguous lineage10.3NOD2, TNF
37nasopharyngitis10.3IFNA2, IL1R1
38pustulosis palmaris et plantaris10.3NOD2, TNF
39hidradenitis suppurativa10.3
40vasculitis10.3
41breast reconstruction10.2
42subcorneal pustular dermatosis10.2
43partial circumpapillary choroid dystrophy10.2IFNA2, MEFV
44placental abruption10.2MEFV, TNF
45rheumatoid arthritis10.2
46hepatitis10.2
47leukemia10.2
48myeloma10.2
49takayasu's arteritis10.2
50psoriasis10.2

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

UMLS symptoms related to Pyoderma Gangrenosum:


breakthrough pain, metastatic pain, tumor pain, cancer pain, cachexia

Drugs & Therapeutics for Pyoderma Gangrenosum

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

(show all 15)
idNameStatusPhaseClinical TrialsCas NumberPubChem Id
1
Minocyclineapproved, investigationalPhase 317310118-90-85281021
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
2Pharmaceutical SolutionsPhase 3, Phase 27004
3Anti-Infective AgentsPhase 317220
4Anti-Bacterial AgentsPhase 39140
5
Tacrolimusapproved, investigationalPhase 21023104987-11-3445643, 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
6Immunologic FactorsPhase 218483
7Antirheumatic AgentsPhase 28496
8Calcineurin InhibitorsPhase 21223
9Immunosuppressive AgentsPhase 210422
10ImmunoglobulinsPhase 24477
11Antibodies, MonoclonalPhase 22413
12AntibodiesPhase 24477
13
Infliximabapproved349170277-31-3
Synonyms:
Ig gamma-1 chain C region
 
Inflectra
Remicade
Remsima
14Dermatologic Agents4555
15Gastrointestinal Agents6401

Interventional clinical trials:

(show all 14)
idNameStatusNCT IDPhase
1Minocycline Therapy for Lung Scarring in Patients With Idiopathic Pulmonary Fibrosis - a Pilot StudyActive, not recruitingNCT00203697Phase 3
2An Efficacy and Safety Study of Gevokizumab in Treating Active Ulcers of Pyoderma GangrenosumTerminatedNCT02326740Phase 3
3An Efficacy and Safety Study of Gevokizumab in Treating Active Ulcers of Pyoderma GangrenosumTerminatedNCT02315417Phase 3
4A 2-Year, Open-Label, Safety Extension Study of Gevokizumab in Subjects With Pyoderma GangrenosumTerminatedNCT02318914Phase 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
9Single-arm Study to Assess a Potential Effect of Anti-IL-17 (Secukinumab) in the Treatment of Pyoderma GangrenosumNot yet recruitingNCT02733094Phase 1, Phase 2
10Safety and Efficacy Study of Humira in Treatment of Pyoderma GangrenosumWithdrawnNCT00730717Phase 2
11Study to Determine the Safety and Efficacy of Adalimumab in the Treatment of Pyoderma GangrenosumWithdrawnNCT00690846Phase 2
12Open Label Study for Adults With Pyoderma Gangrenosum and Inflammatory Bowel DiseaseCompletedNCT00791557
13Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin DiseasesRecruitingNCT01952275
14Schnitzler 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, T cells, Lung, Heart, Bone

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:00053977.7IL1R1, MEFV, NLRP3, NOD2, PSTPIP1, TNF
2MP:00053876.8IL1R1, MEFV, MMP10, NLRP3, NOD2, PSTPIP1

Publications for Pyoderma Gangrenosum

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Articles related to Pyoderma Gangrenosum:

(show top 50)    (show all 657)
idTitleAuthorsYear
1
Successful treatment of large refractory pyoderma gangrenosum-like presentation of granulomatosis with polyangiitis with rituximab. (27126548)
2016
2
Misdiagnosed Periocular Pyoderma Gangrenosum Requiring Ectropion Repair With Development of Second Lesion of Pyoderma Gangrenosum at Graft Site. (26934168)
2016
3
Pyoderma gangrenosum of the breast in a patient with a history of silicone augmentation mastopexy and suction-assisted lipectomy of the trunk. (27051770)
2015
4
Granulomatous pyoderma gangrenosum of the genitalia in the absence of Crohn's disease. (26392658)
2015
5
Pyoderma gangrenosum secondary to azacitidine in myelodysplastic syndrome. (25765235)
2015
6
The Genetics of Pyoderma Gangrenosum and Implications for Treatment: A Systematic Review. (25350484)
2014
7
Recurrent pyoderma gangrenosum after cesarean delivery successfully treated with vacuum-assisted closure and split thickness skin graft: A case report. (25370183)
2014
8
Pyoderma gangrenosum with renal and splenic impairment - case report. (24346905)
2013
9
Penile pyoderma gangrenosum. (24339469)
2013
10
Letter to the editor: Temporary renal insufficiency associated with topical tacrolimus treatment of multilocal pyoderma gangrenosum. (24133568)
2013
11
Pyoderma gangrenosum in a newborn - case report. (24346911)
2013
12
Dermacase. Can you identify this condition? Pyoderma gangrenosum. (23585606)
2013
13
Auricular pyoderma gangrenosum associated with Crohn's disease. (22585655)
2012
14
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
15
Refractory pyoderma gangrenosum associated with ulcerative colitis successfully treated with infliximab. (22301049)
2012
16
Pyoderma gangrenosum in breast reconstruction. (22747349)
2012
17
Incidence, mortality, and disease associations of pyoderma gangrenosum in the United Kingdom: a retrospective cohort study. (22534879)
2012
18
Pyoderma gangrenosum in a patient with primary cutaneous peripheral T-cell lymphoma, not otherwise specified. (23228951)
2012
19
Penile pyoderma gangrenosum successfully treated with topical Imiquimod. (22021975)
2011
20
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
21
Hyperbaric oxygen therapy as an adjuvant treatment for pyoderma gangrenosum. (22281912)
2011
22
Pyoderma gangrenosum following trauma of the knee: a case of pathergy and review of orthopaedic cases. (20638661)
2011
23
Ciprofloxacin induced pyoderma gangrenosum. (23130244)
2011
24
Pyoderma gangrenosum of the breast after bilateral simple mastectomies for ductal carcinoma in situ. (21944335)
2011
25
Pyoderma gangrenosum associated with chronic idiopathic myelofibrosis after coronary artery bypass graft surgery. (19846444)
2010
26
Beclometasone inhaler used to treat pyoderma gangrenosum. (20500206)
2010
27
A case of pyoderma gangrenosum with ulcerative colitis treated with mesalazine. (21165212)
2010
28
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
29
Adalimumab treatment for peristomal pyoderma gangrenosum associated with Crohn's disease. (18942748)
2009
30
Early diagnosis and early corticosteroid administration improves healing of peristomal pyoderma gangrenosum in inflammatory bowel disease. (19279428)
2009
31
Seat belt pyoderma gangrenosum: minor pressure as a causative factor. (18837131)
2008
32
Pyoderma gangrenosum of the scalp following hair highlights in a postpartum patient. (18086612)
2008
33
An open-label pilot study of alefacept for the treatment of pyoderma gangrenosum. (18384543)
2008
34
A novel therapy for the treatment of pyoderma gangrenosum. (18935734)
2008
35
Pyoderma gangrenosum in the head and neck. (17224531)
2007
36
Negative pressure dressing in the management of pyoderma gangrenosum ulcer. (17938337)
2007
37
Pulmonary manifestations of Pyoderma gangrenosum. (17555913)
2007
38
Pyoderma gangrenosum in an infant. (16918628)
2006
39
The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum. (16415387)
2006
40
A case of pyoderma gangrenosum responding to high-dose intravenous immunoglobulin therapy. (16863619)
2006
41
An exuberant inflammatory response to E coli: implications for the pathogenesis of ulcerative colitis and pyoderma gangrenosum. (17047113)
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
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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GO Terms for genes affiliated with Pyoderma Gangrenosum

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

(show all 9)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of cysteine-type endopeptidase activity involved in apoptotic processGO:004328010.2NLRP3, TNF
2positive regulation of interleukin-1 beta secretionGO:00507189.7NLRP3, NOD2
3nucleotide-binding domain, leucine rich repeat containing receptor signaling pathwayGO:00358729.6NLRP3, NOD2
4protein oligomerizationGO:00512599.6NLRP3, NOD2
5defense response to bacteriumGO:00427429.3NOD2, TNF
6cell surface receptor signaling pathwayGO:00071669.2IFNA2, IL1R1, TNF
7positive regulation of NF-kappaB transcription factor activityGO:00510928.9NLRP3, NOD2, TNF
8inflammatory responseGO:00069548.6IFNA2, NLRP3, PSTPIP1, TNF
9defense responseGO:00069528.6IFNA2, NLRP3, NOD2, TNF

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