MCID: PYD001
MIFTS: 55

Pyoderma Gangrenosum

Categories: Rare diseases, Skin diseases, Bone diseases

Aliases & Classifications for Pyoderma Gangrenosum

MalaCards integrated aliases for Pyoderma Gangrenosum:

Name: Pyoderma Gangrenosum 12 72 49 55 51 41 14 69

Characteristics:

Orphanet epidemiological data:

55
pyoderma gangrenosum
Prevalence: 1-9/100000 (United States),1-9/1000000 (United Kingdom),1-9/1000000 (Spain),1-9/1000000 (Worldwide); Age of onset: Adolescent,Adult,Childhood,Elderly; Age of death: normal life expectancy;

Classifications:



External Ids:

Disease Ontology 12 DOID:8553
ICD10 32 L88
ICD9CM 34 686.01
MeSH 41 D017511
SNOMED-CT 64 74578003
Orphanet 55 ORPHA48104
MESH via Orphanet 42 D017511
UMLS via Orphanet 70 C0085652
ICD10 via Orphanet 33 L88
UMLS 69 C0085652

Summaries for Pyoderma Gangrenosum

NIH Rare Diseases : 49 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, vasculitis, leukemia, and other conditions. Each year in the United States, pyoderma gangrenosum occurs in about 1 person per 100,000 people. Pyoderma gangrenosum belongs to a group of autoinflammatory skin diseases called neutrophilic dermatoses. Neutrophils are a type of white blood cell or leukocyte which form an early line of defense against bacterial infections. Ulcerations associated with pyoderma gangrenosum may occur after trauma or injury to the skin, a process called pathergy. Treatment involves wound care and the use of anti-inflammatory agents, including antibiotics, corticosteroids, immunosuppressants, and biologics.  Last updated: 8/15/2016

MalaCards based summary : Pyoderma Gangrenosum is related to ileocolitis and hidradenitis, and has symptoms including atrophic scars, rheumatoid arthritis and fever. An important gene associated with Pyoderma Gangrenosum is PSTPIP1 (Proline-Serine-Threonine Phosphatase Interacting Protein 1), and among its related pathways/superpathways are Innate Immune System and Cytokine Signaling in Immune system. The drugs Minocycline and Adalimumab have been mentioned in the context of this disorder. Affiliated tissues include skin, neutrophil and lung, and related phenotype is immune system.

Wikipedia : 72 Pyoderma gangrenosum is a condition that causes tissue to become necrotic, causing deep ulcers that... more...

Related Diseases for Pyoderma Gangrenosum

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

(show top 50) (show all 274)
# Related Disease Score Top Affiliating Genes
1 ileocolitis 32.4 NOD2 TNF
2 hidradenitis 31.0 NOD2 TNF
3 colitis 30.9 CXCL8 NOD2 TNF
4 ulcerative colitis 30.9 CXCL8 NOD2 TNF
5 hidradenitis suppurativa 30.9 NOD2 TNF
6 vasculitis 30.6 IFNA2 MEFV TNF
7 scleritis 30.5 ITGB2 TNF
8 fasciitis 30.5 CXCL8 TNF
9 arthritis 30.5 CXCL8 NOD2 TNF
10 pyoderma 30.4 CXCL8 IFNA2 ITGB2 MEFV MMP10 NOD2
11 sapho syndrome 30.4 CXCL8 NOD2
12 inflammatory bowel disease 30.3 CXCL8 NOD2 PSTPIP1 TNF
13 pouchitis 30.3 CXCL8 NOD2
14 polyarteritis nodosa 30.1 IFNA2 MEFV
15 wegener granulomatosis 30.1 CXCL8 ITGB2 TNF
16 acute respiratory distress syndrome 29.8 CXCL8 TNF
17 mooren's ulcer 29.5 ITGB2 NLRP3
18 aphthous stomatitis 29.4 MEFV NLRP3 TNF
19 pyogenic arthritis, pyoderma gangrenosum and acne 12.7
20 pyogenic sterile arthritis, pyoderma gangrenosum, and acne 12.5
21 pyoderma gangrenosum-acne-suppurative hidradenitis syndrome 12.3
22 corticosteroid-sensitive aseptic abscesses 11.5
23 polydactyly, postaxial, type a1 11.2
24 sweat gland disease 10.4 PSTPIP1 TNF
25 systemic onset juvenile idiopathic arthritis 10.4 MEFV TNF
26 autoinflammation, panniculitis, and dermatosis syndrome 10.4 MEFV TNF
27 crohn's disease 10.4
28 erysipelas 10.4 MEFV TNF
29 granulomatous dermatitis 10.4 NOD2 TNF
30 myelodysplastic syndrome 10.4
31 tungiasis 10.4 CXCL8 TNF
32 streptococcal toxic-shock syndrome 10.4 CXCL8 TNF
33 inflammatory bowel disease 3 10.3 NOD2 TNF
34 lymphoma 10.3
35 trichuriasis 10.3 CXCL8 TNF
36 haemophilus influenzae 10.3 CXCL8 TNF
37 streptococcal meningitis 10.3 NOD2 TNF
38 clonorchiasis 10.3 CXCL8 TNF
39 meconium aspiration syndrome 10.3 CXCL8 TNF
40 sebaceous gland disease 10.3 CXCL8 PSTPIP1
41 dental pulp disease 10.3 CXCL8 TNF
42 louse-borne relapsing fever 10.3 CXCL8 TNF
43 transverse myelitis 10.3 CXCL8 TNF
44 tuberculous meningitis 10.3 CXCL8 TNF
45 pleural tuberculosis 10.3 CXCL8 TNF
46 adult-onset still's disease 10.3 MEFV TNF
47 pulpitis 10.3 CXCL8 TNF
48 granulocytopenia 10.2 ITGB2 TNF
49 cytomegalovirus infection 10.2 CXCL8 TNF
50 middle ear disease 10.2 CXCL8 TNF

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



Diseases related to Pyoderma Gangrenosum

Symptoms & Phenotypes for Pyoderma Gangrenosum

Human phenotypes related to Pyoderma Gangrenosum:

55 31 (show all 15)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 atrophic scars 55 31 frequent (33%) Frequent (79-30%) HP:0001075
2 rheumatoid arthritis 55 31 frequent (33%) Frequent (79-30%) HP:0001370
3 fever 55 31 hallmark (90%) Very frequent (99-80%) HP:0001945
4 inflammation of the large intestine 55 31 frequent (33%) Frequent (79-30%) HP:0002037
5 arthralgia 55 31 hallmark (90%) Very frequent (99-80%) HP:0002829
6 myelodysplasia 55 31 frequent (33%) Frequent (79-30%) HP:0002863
7 myalgia 55 31 hallmark (90%) Very frequent (99-80%) HP:0003326
8 abnormal blistering of the skin 55 31 occasional (7.5%) Occasional (29-5%) HP:0008066
9 increased antibody level in blood 55 31 frequent (33%) Frequent (79-30%) HP:0010702
10 myeloid leukemia 55 31 frequent (33%) Frequent (79-30%) HP:0012324
11 myositis 55 31 hallmark (90%) Very frequent (99-80%) HP:0100614
12 papule 55 31 hallmark (90%) Very frequent (99-80%) HP:0200034
13 skin vesicle 55 31 occasional (7.5%) Occasional (29-5%) HP:0200037
14 pustule 55 31 frequent (33%) Frequent (79-30%) HP:0200039
15 skin ulcer 55 31 hallmark (90%) Very frequent (99-80%) HP:0200042

MGI Mouse Phenotypes related to Pyoderma Gangrenosum:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 immune system MP:0005387 9.17 ITGB2 MEFV MMP10 NLRP3 NOD2 PSTPIP1

Drugs & Therapeutics for Pyoderma Gangrenosum

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

(show all 16)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Minocycline Approved, Investigational Phase 3 10118-90-8 5281021
2
Adalimumab Approved Phase 3,Phase 2 331731-18-1 16219006
3 Anti-Bacterial Agents Phase 3
4 Anti-Infective Agents Phase 3
5 Pharmaceutical Solutions Phase 3,Phase 2
6 Antirheumatic Agents Phase 3,Phase 2
7 Anti-Inflammatory Agents Phase 3,Phase 2
8
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492
9 Dermatologic Agents Phase 2
10 Antibodies, Monoclonal Phase 2,Phase 1
11 Immunoglobulins Phase 2,Phase 1
12 Immunosuppressive Agents Phase 2
13 Antibodies Phase 2,Phase 1
14 Calcineurin Inhibitors Phase 2
15
Infliximab Approved 170277-31-3
16 Gastrointestinal Agents

Interventional clinical trials:

(show all 17)

# Name Status NCT ID Phase Drugs
1 Minocycline Therapy for Lung Scarring in Patients With Idiopathic Pulmonary Fibrosis - a Pilot Study Unknown status NCT00203697 Phase 3 minocycline
2 A Study Assessing the Efficacy and Safety of Adalimumab in Active Ulcer(s) of Pyoderma Gangrenosum in Participants in Japan Recruiting NCT03311464 Phase 3 adalimumab
3 An Efficacy and Safety Study of Gevokizumab in Treating Active Ulcers of Pyoderma Gangrenosum Terminated NCT02326740 Phase 3 gevokizumab;Placebo;gevokizumab open-label
4 An Efficacy and Safety Study of Gevokizumab in Treating Active Ulcers of Pyoderma Gangrenosum Terminated NCT02315417 Phase 3 gevokizumab;Placebo;gevokizumab open-label
5 A 2-Year, Open-Label, Safety Extension Study of Gevokizumab in Subjects With Pyoderma Gangrenosum Terminated NCT02318914 Phase 3 gevokizumab
6 Proof of Concept Study of Gevokizumab in the Treatment of Pyoderma Gangrenosum Completed NCT01882504 Phase 2 gevokizumab
7 A Phase II Open Label Study of Xilonix in Subjects With Pyoderma Gangrenosum Completed NCT01965613 Phase 2
8 Canakinumab for Pyoderma Gangrenosum Completed NCT01302795 Phase 2 Canakinumab
9 Topical Tacrolimus 0.1% Ointment For Treatment Of Cutaneous Crohn's Disease Completed NCT01233570 Phase 2 Tacrolimus
10 An Open-Label, Proof-of-Concept Study of Ixekizumab in the Treatment of Pyoderma Gangrenosum Recruiting NCT03137160 Phase 2
11 Single-arm Study to Assess a Potential Effect of Anti-IL-17 (Secukinumab) in the Treatment of Pyoderma Gangrenosum Recruiting NCT02733094 Phase 1, Phase 2 Secukinumab
12 Efficacy and Safety of APD334 in Patients With Pyoderma Gangrenosum Recruiting NCT03072953 Phase 2 APD334
13 Safety and Efficacy Study of Humira in Treatment of Pyoderma Gangrenosum Withdrawn NCT00730717 Phase 2 Humira;Humira
14 Study to Determine the Safety and Efficacy of Adalimumab in the Treatment of Pyoderma Gangrenosum Withdrawn NCT00690846 Phase 2 adalimumab
15 Open Label Study for Adults With Pyoderma Gangrenosum and Inflammatory Bowel Disease Completed NCT00791557 Infliximab
16 Schnitzler Syndrome: Clinical Study, Physiopathological and Search for Genetic Factors Completed NCT00933296
17 Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases Recruiting NCT01952275

Search NIH Clinical Center for Pyoderma Gangrenosum

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Cochrane evidence based reviews: pyoderma gangrenosum

Genetic Tests for Pyoderma Gangrenosum

Anatomical Context for Pyoderma Gangrenosum

MalaCards organs/tissues related to Pyoderma Gangrenosum:

38
Skin, Neutrophil, Lung, T Cells, Bone, Myeloid, Monocytes

Publications for Pyoderma Gangrenosum

Articles related to Pyoderma Gangrenosum:

(show top 50) (show all 747)
# Title Authors Year
1
A systematic review of pyoderma gangrenosum with pulmonmary involvement: clinical presentation, diagnosis, and management. ( 29377399 )
2018
2
The impact of underlying disease state on outcomes in patients with pyoderma gangrenosum: A national survey. ( 29438762 )
2018
3
Hyperbaric oxygen therapy for pyoderma gangrenosum associated with ulcerative colitis. ( 29422812 )
2018
4
Pyoderma gangrenosum successfully treated with topical tacrolimus. ( 29368849 )
2018
5
Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts. ( 29450466 )
2018
6
A case of pyoderma gangrenosum, acne, hidradenitis suppurativa (PASH) syndrome associated with diabetes mellitus type 1 treated with adalimumab. ( 29368847 )
2018
7
Cutaneous Diphtheria Mimicking Pyoderma Gangrenosum. ( 29282460 )
2018
8
Management of Facial Pyoderma Gangrenosum Using Platelet-Rich Fibrin: A Technical Report. ( 29425752 )
2018
9
Diagnosis uPGrade-Advances in Pyoderma Gangrenosum. ( 29450448 )
2018
10
Pyoderma gangrenosum in the genital area: successful treatment using adalimumab. ( 29378677 )
2018
11
Pyoderma Gangrenosum Under Dabrafenib and Trametinib for Metastatic Melanoma. ( 29362816 )
2018
12
Pyoderma gangrenosum: a review of pathogenesis and treatment. ( 29406827 )
2018
13
<i>LPIN2</i> gene mutation in a patient with overlapping neutrophilic disease (pyoderma gangrenosum and aseptic abscess syndrome). ( 29387759 )
2018
14
The PARACELSUS score: A novel diagnostic tool for pyoderma gangrenosum. ( 29388188 )
2018
15
Paraneoplastic Pyoderma Gangrenosum Associated with Rectal Adenocarcinoma. ( 29386837 )
2018
16
The Validity of the Diagnostic Code for Pyoderma Gangrenosum in an Electronic Database. ( 29451671 )
2018
17
Suppression of pathergy in pyoderma gangrenosum with infliximab allowing for successful tendon debridement. ( 29379860 )
2018
18
Successful therapy of Pyoderma gangrenosum with a JAK2 inhibitor. ( 29451690 )
2018
19
Pulmonary nodules and nodular scleritis in a teenager with superficial granulomatous pyoderma gangrenosum. ( 29243307 )
2018
20
The Association of Age With Clinical Presentation and Comorbidities of Pyoderma Gangrenosum. ( 29450453 )
2018
21
Granulomatosis with Polyangiitis Presenting with Pyoderma Gangrenosum-Like Ulceration and Negative Cytoplasmic Antineutrophilic Cytoplasmic Antibodies in a Child. ( 28884919 )
2017
22
Bullous Pyoderma Gangrenosum Associated with Hairy Cell Leukemia and Its Complete Response to Cladribine Therapy. ( 28596664 )
2017
23
Successful use of etoposide for pyoderma gangrenosum associated with myelodysplastic syndrome and trisomy 8: cytokine profiles during treatment. ( 28943508 )
2017
24
Pyoderma gangrenosum affecting the eye, orbit, and adnexa. A review. ( 28862478 )
2017
25
Pyoderma Gangrenosum in a Patient with X-Linked Agammaglobulinemia. ( 28761297 )
2017
26
Risk of Developing Pyoderma Gangrenosum after Procedures in Patients with a Known History of Pyoderma Gangrenosum - A Retrospective Analysis. ( 28947285 )
2017
27
Extensive Pyoderma Gangrenosum: A Challenging Diagnosis and Literature Review of Management. ( 28944125 )
2017
28
Iliopsoas and intraperitoneal abscesses associated with pyoderma gangrenosum. ( 28869344 )
2017
29
SAPHO syndrome associated with hidradenitis suppurativa and pyoderma gangrenosum successfully treated with adalimumab and methotrexate: a case report and review of the literature. ( 28884797 )
2017
30
Successful treatment of a patient with myelodysplastic syndrome accompanied by pyoderma gangrenosum and BehAset's disease using allogeneic stem cell transplantation. ( 29333411 )
2017
31
An Unusual Association of Chronic Recurrent Multifocal Osteomyelitis, Pyoderma Gangrenosum, and Takayasu Arteritis. ( 28042129 )
2017
32
Pyoderma Gangrenosum as the Initial Manifestation of Small Cell Lung Cancer with Dermatomyositis. ( 28836564 )
2017
33
Reconstructive microsurgical approach for the treatment of pyoderma gangrenosum. ( 28918934 )
2017
34
Non-response to Interleukin-1 Antagonist Canakinumab in Two Patients with Refractory Pyoderma Gangrenosum and Hidradenitis Suppurativa. ( 29344326 )
2017
35
Pyoderma Gangrenosum: A Current Problem as Much as an Unknown One. ( 28915774 )
2017
36
Sporotrichosis masquerading as pyoderma gangrenosum. ( 28627095 )
2017
37
Pyoderma Gangrenosum: An Uncommon Case Report and Review of the Literature. ( 28933699 )
2017
38
Pyoderma gangrenosum-like ulcer caused by Helicobacter cinaedi in a patient with hypogammaglobulinemia. ( 28815770 )
2017
39
Image Gallery: Pyoderma gangrenosum in a patient with myelodysplastic syndrome treated with azacitidine. ( 28940271 )
2017
40
Topical Medical Cannabis (TMC): A new treatment for wound pain-Three cases of Pyoderma Gangrenosum. ( 28818631 )
2017
41
Painful linear ulcers: A case of cutaneous sporotrichosis mimicking pyoderma gangrenosum. ( 29264381 )
2017
42
Pyoderma gangrenosum, acne, and suppurative hidradenitis syndrome in end-stage renal disease successfully treated with adalimumab. ( 29447652 )
2017
43
A case of eosinophilic fasciitis associated with pyoderma gangrenosum. ( 28150344 )
2017
44
Classic Ulcerative Pyoderma Gangrenosum Is a T Cell-Mediated Disease Targeting Follicular Adnexal Structures: A Hypothesis Based on Molecular and Clinicopathologic Studies. ( 29379508 )
2017
45
Successful treatment of pyoderma gangrenosum with anakinra in a patient with Wiskott-Aldrich syndrome. ( 29214738 )
2017
46
Comparison of pyoderma gangrenosum and hypertensive ischemic leg ulcer Martorell in a Swiss cohort. ( 28832960 )
2017
47
Pyoderma gangrenosum associated with Crohn's disease in a Malawian teenage boy: case report and review of literature. ( 28767000 )
2017
48
Pyoderma gangrenosum in pregnancy successfully treated with infliximab and prednisone. ( 28879220 )
2017
49
Pyoderma Gangrenosum after Breast Mastectomy and Primary Rectus Abdominis Flap Reconstruction. ( 28871582 )
2017
50
Pyoderma gangrenosum in a patient with chronic granulomatous disease: A case report. ( 28767612 )
2017

Variations for Pyoderma Gangrenosum

Expression for Pyoderma Gangrenosum

Search GEO for disease gene expression data for Pyoderma Gangrenosum.

Pathways for Pyoderma Gangrenosum

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

(show all 20)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.69 CXCL8 IFNA2 ITGB2 MEFV NLRP3 NOD2
2
Show member pathways
13.1 CXCL8 IFNA2 ITGB2 NOD2 TNF
3
Show member pathways
12.6 CXCL8 IFNA2 ITGB2 TNF
4
Show member pathways
12.54 CXCL8 IFNA2 NLRP3 TNF
5
Show member pathways
12 IFNA2 NLRP3 TNF
6
Show member pathways
11.99 CXCL8 IFNA2 MEFV NLRP3 NOD2 PSTPIP1
7
Show member pathways
11.9 CXCL8 IFNA2 TNF
8 11.88 IFNA2 ITGB2 NOD2 TNF
9 11.82 CXCL8 IFNA2 TNF
10 11.71 CXCL8 ITGB2 TNF
11 11.62 CXCL8 ITGB2 TNF
12 11.56 CXCL8 ITGB2 TNF
13
Show member pathways
11.52 MEFV NLRP3 NOD2 PSTPIP1
14 11.32 CXCL8 ITGB2 TNF
15 11.23 CXCL8 ITGB2 TNF
16 11.14 CXCL8 ITGB2 NLRP3 TNF
17 11.05 MEFV NLRP3 NOD2
18 10.96 CXCL8 TNF
19 10.93 NOD2 TNF
20 10.82 CXCL8 TNF

GO Terms for Pyoderma Gangrenosum

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

(show all 18)
# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.84 MEFV NLRP3 NOD2 PSTPIP1
2 innate immune response GO:0045087 9.8 IFNA2 MEFV NLRP3 NOD2 PSTPIP1
3 cellular response to lipopolysaccharide GO:0071222 9.69 CXCL8 NLRP3 TNF
4 negative regulation of inflammatory response GO:0050728 9.67 MEFV MVK NLRP3
5 regulation of inflammatory response GO:0050727 9.65 NLRP3 NOD2 TNF
6 receptor internalization GO:0031623 9.58 CXCL8 ITGB2
7 positive regulation of interleukin-8 production GO:0032757 9.57 NOD2 TNF
8 positive regulation of NIK/NF-kappaB signaling GO:1901224 9.56 NOD2 TNF
9 positive regulation of interleukin-1 beta secretion GO:0050718 9.55 NLRP3 NOD2
10 embryonic digestive tract development GO:0048566 9.52 CXCL8 TNF
11 positive regulation of type 2 immune response GO:0002830 9.46 NLRP3 NOD2
12 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.46 ITGB2 NLRP3 NOD2 TNF
13 cellular response to peptidoglycan GO:0071224 9.43 NLRP3 NOD2
14 cellular extravasation GO:0045123 9.37 ITGB2 TNF
15 defense response GO:0006952 9.35 CXCL8 IFNA2 NLRP3 NOD2 TNF
16 detection of biotic stimulus GO:0009595 9.32 NLRP3 NOD2
17 cytokine secretion involved in immune response GO:0002374 9.26 NLRP3 NOD2
18 inflammatory response GO:0006954 9.17 CXCL8 IFNA2 ITGB2 MEFV NLRP3 PSTPIP1

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

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.35 MEFV MVK NLRP3 PSTPIP1 TNF
2 cytokine activity GO:0005125 9.33 CXCL8 IFNA2 TNF
3 peptidoglycan binding GO:0042834 8.62 NLRP3 NOD2

Sources for Pyoderma Gangrenosum

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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