MCID: PYD001
MIFTS: 51

Pyoderma Gangrenosum malady

Categories: Rare diseases, Skin diseases, Bone diseases

Aliases & Classifications for Pyoderma Gangrenosum

Aliases & Descriptions for Pyoderma Gangrenosum:

Name: Pyoderma Gangrenosum 12 50 56 52 42 14 69

Characteristics:

Orphanet epidemiological data:

56
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 33 L88
ICD9CM 35 686.01
MeSH 42 D017511
SNOMED-CT 64 74578003
Orphanet 56 ORPHA48104
MESH via Orphanet 43 D017511
UMLS via Orphanet 70 C0085652
ICD10 via Orphanet 34 L88
UMLS 69 C0085652

Summaries for Pyoderma Gangrenosum

NIH Rare Diseases : 50 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 collagenous colitis and rheumatoid arthritis, and has symptoms including arthralgia, myalgia 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 Anti-Bacterial Agents have been mentioned in the context of this disorder. Affiliated tissues include skin, neutrophil and breast, and related phenotypes are hematopoietic system and immune system

Wikipedia : 71 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 240)
id Related Disease Score Top Affiliating Genes
1 collagenous colitis 30.0 CXCL8 NOD2 TNF
2 rheumatoid arthritis 29.9 CXCL8 IL1R1 ITGB2 TNF
3 prostatitis 29.6 IL1R1 NLRP3
4 invasive aspergillosis 29.6 MEFV NLRP3
5 skin conditions 29.4 CXCL8 NLRP3 TNF
6 pyogenic sterile arthritis, pyoderma gangrenosum, and acne 12.4
7 pyogenic arthritis, pyoderma gangrenosum and acne 12.3
8 pyoderma gangrenosum-acne-suppurative hidradenitis syndrome 12.1
9 pyoderma 11.7
10 crohn's disease 11.5
11 ileocolitis 11.1
12 polydactyly, postaxial, types a1 and b 11.1
13 colitis 10.5
14 ulcerative colitis 10.5
15 acne 10.4
16 inflammatory bowel disease 10.4
17 arthritis 10.4
18 radial ray agenesis 10.3 IL1R1 PSTPIP1
19 nemaline myopathy 1, autosomal dominant or recessive 10.3 PSTPIP1 TNF
20 hidradenitis 10.2
21 myeloid neoplasms associated with pdgfrb rearrangement 10.2 MEFV TNF
22 tau syndrome 10.2 MEFV TNF
23 lymphoma 10.2
24 dermatosis papulosa nigra 10.2 NOD2 TNF
25 myelodysplastic syndrome 10.2
26 amyotrophic lateral sclerosis type 5 10.2 NOD2 TNF
27 tetanus 10.2 MEFV TNF
28 psoriatic arthritis 10.2 NOD2 TNF
29 conidiobolomycosis 10.2 CXCL8 TNF
30 thymic neuroendocrine carcinoma 10.2 CXCL8 TNF
31 female reproductive system disease 10.2 NOD2 TNF
32 coronary arterial fistulas 10.2 CXCL8 NOD2
33 acute gonococcal cystitis 10.2 CXCL8 NOD2
34 handl syndrome 10.2 CXCL8 TNF
35 nephronophthisis 1, juvenile 10.1 CXCL8 TNF
36 plantar wart 10.1 CXCL8 TNF
37 urethral intrinsic sphincter deficiency 10.1 ITGB2 TNF
38 esophagus lymphoma 10.1 CXCL8 TNF
39 benign neonatal seizures 10.1 IL1R1 MEFV TNF
40 hidradenitis suppurativa 10.1
41 vasculitis 10.1
42 functional gastric disease 10.1 IL1R1 NOD2 TNF
43 esophageal candidiasis 10.1 IL1R1 NOD2 TNF
44 ulcerative proctitis 10.1 CXCL8 TNF
45 breast reconstruction 10.1
46 hepatitis 10.1
47 subcorneal pustular dermatosis 10.1
48 bare lymphocyte syndrome, type i 10.1 CXCL8 IL1R1 TNF
49 dermatomycosis 10.1 MEFV TNF
50 strawberry gallbladder 10.1 CXCL8 MEFV 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:

56 32 (show all 15)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 arthralgia 56 32 Very frequent (99-80%) HP:0002829
2 myalgia 56 32 Very frequent (99-80%) HP:0003326
3 fever 56 32 Very frequent (99-80%) HP:0001945
4 myelodysplasia 56 32 Frequent (79-30%) HP:0002863
5 abnormal blistering of the skin 56 32 Occasional (29-5%) HP:0008066
6 inflammation of the large intestine 56 32 Frequent (79-30%) HP:0002037
7 skin ulcer 56 32 Very frequent (99-80%) HP:0200042
8 papule 56 32 Very frequent (99-80%) HP:0200034
9 myositis 56 32 Very frequent (99-80%) HP:0100614
10 skin vesicle 56 32 Occasional (29-5%) HP:0200037
11 increased antibody level in blood 56 32 Frequent (79-30%) HP:0010702
12 rheumatoid arthritis 56 32 Frequent (79-30%) HP:0001370
13 pustule 56 32 Frequent (79-30%) HP:0200039
14 atrophic scars 56 32 Frequent (79-30%) HP:0001075
15 myeloid leukemia 56 32 Frequent (79-30%) HP:0012324

MGI Mouse Phenotypes related to Pyoderma Gangrenosum:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.7 NLRP3 NOD2 PSTPIP1 TNF IL1R1 ITGB2
2 immune system MP:0005387 9.56 IL1R1 ITGB2 MEFV MMP10 NLRP3 NOD2
3 skeleton MP:0005390 9.1 IL1R1 ITGB2 MEFV NLRP3 NOD2 TNF

Drugs & Therapeutics for Pyoderma Gangrenosum

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

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

Interventional clinical trials:

(show all 16)
id Name Status NCT ID Phase
1 Minocycline Therapy for Lung Scarring in Patients With Idiopathic Pulmonary Fibrosis - a Pilot Study Unknown status NCT00203697 Phase 3
2 An Efficacy and Safety Study of Gevokizumab in Treating Active Ulcers of Pyoderma Gangrenosum Terminated NCT02326740 Phase 3
3 An Efficacy and Safety Study of Gevokizumab in Treating Active Ulcers of Pyoderma Gangrenosum Terminated NCT02315417 Phase 3
4 A 2-Year, Open-Label, Safety Extension Study of Gevokizumab in Subjects With Pyoderma Gangrenosum Terminated NCT02318914 Phase 3
5 Proof of Concept Study of Gevokizumab in the Treatment of Pyoderma Gangrenosum Completed NCT01882504 Phase 2
6 A Phase II Open Label Study of Xilonix in Subjects With Pyoderma Gangrenosum Completed NCT01965613 Phase 2
7 Canakinumab for Pyoderma Gangrenosum Completed NCT01302795 Phase 2
8 Topical Tacrolimus 0.1% Ointment For Treatment Of Cutaneous Crohn's Disease Completed NCT01233570 Phase 2
9 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
10 An Open-Label, Proof-of-Concept Study of Ixekizumab in the Treatment of Pyoderma Gangrenosum Recruiting NCT03137160 Phase 2
11 Efficacy and Safety of APD334 in Patients With Pyoderma Gangrenosum Recruiting NCT03072953 Phase 2
12 Safety and Efficacy Study of Humira in Treatment of Pyoderma Gangrenosum Withdrawn NCT00730717 Phase 2
13 Study to Determine the Safety and Efficacy of Adalimumab in the Treatment of Pyoderma Gangrenosum Withdrawn NCT00690846 Phase 2
14 Schnitzler Syndrome: Clinical Study, Physiopathological and Search for Genetic Factors Unknown status NCT00933296
15 Open Label Study for Adults With Pyoderma Gangrenosum and Inflammatory Bowel Disease Completed NCT00791557
16 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 48 :


Cochrane evidence based reviews: pyoderma gangrenosum

Genetic Tests for Pyoderma Gangrenosum

Anatomical Context for Pyoderma Gangrenosum

MalaCards organs/tissues related to Pyoderma Gangrenosum:

39
Skin, Neutrophil, Breast, T Cells, Bone, Myeloid, Lung

Publications for Pyoderma Gangrenosum

Articles related to Pyoderma Gangrenosum:

(show top 50) (show all 698)
id Title Authors Year
1
An Unusual Association of Chronic Recurrent Multifocal Osteomyelitis, Pyoderma Gangrenosum, and Takayasu Arteritis. ( 28042129 )
2017
2
Pyoderma Gangrenosum, Acne and Suppurative Hidradenitis Syndrome Treated with Granulocyte and Monocyte Adsorption Apheresis. ( 27384061 )
2016
3
Treatment of refractory pyoderma gangrenosum with infliximab in a 17-month-old boy. ( 27617462 )
2016
4
Pyoderma gangrenosum and necrotizing fasciitis-like opportunistic invasive cutaneous fungal infection. ( 27126743 )
2016
5
Clinical features, causes, treatments, and outcomes of peristomal pyoderma gangrenosum (PPG) in 44 patients: The Mayo Clinic experience, 1996 through 2013. ( 27473454 )
2016
6
Biologics for the treatment of pyoderma gangrenosum in ulcerative colitis. ( 27799888 )
2016
7
Facial Pyoderma Gangrenosum Presenting With Cranial Nerve VII Palsy and Cicatricial Ectropion. ( 27893584 )
2016
8
Pediatric pyoderma gangrenosum: is it just big wounds on little adults? ( 27916349 )
2016
9
High level of serum human interleukin-18 in a patient with pyogenic arthritis, pyoderma gangrenosum and acne syndrome. ( 27505688 )
2016
10
Pyoderma gangrenosum of the eyelid. ( 27085278 )
2016
11
Postoperative Pyoderma Gangrenosum: AA Clinical Review of Published Cases. ( 27489052 )
2016
12
Gene Therapy for Pyoderma Gangrenosum: Optimal Transfection Conditions and Effect of Drugs on Gene Delivery in the HaCaT Cell Line Using Cationic Liposomes. ( 27159975 )
2016
13
Post Surgical Pyoderma Gangrenosum in flap surgery: diagnostic clues and treatment recommendations. ( 27938245 )
2016
14
Pyoderma gangrenosum with extensive pulmonary involvement. ( 27658239 )
2016
15
Pyoderma gangrenosum and its syndromic forms: Evidence for a link with autoinflammation. ( 27106250 )
2016
16
Successful Treatment of Pyoderma Gangrenosum after Augmentation Mastopexy Using Vacuum Therapy. ( 27975008 )
2016
17
Severe pyoderma gangrenosum in association with a flame burn. ( 27583260 )
2016
18
Intralesional methotrexate as an adjuvant treatment for pyoderma gangrenosum: A case report. ( 27451930 )
2016
19
Severe Bilateral Lower Extremity Pyoderma Gangrenosum. ( 27980702 )
2016
20
Pyoderma gangrenosum after trauma in a dog. ( 27108868 )
2016
21
Successful Treatment of Pyoderma Gangrenosum with Cryoglobulinemia and Hepatitis C. ( 27345376 )
2016
22
Delayed diagnosis of bullous pyoderma gangrenosum with acute myelogenous leukemia. ( 27811462 )
2016
23
Autoinflammation in pyoderma gangrenosum and its syndromic form PASH. ( 27943240 )
2016
24
Pyoderma gangrenosum associated with mantle cell lymphoma. ( 27559522 )
2016
25
Pyoderma gangrenosum mimicking mediastinitis after cardiac surgery: a case study. ( 27286670 )
2016
26
Treatment of pyoderma gangrenosum with thalidomide in a myelodysplastic syndrome case. ( 27051318 )
2016
27
Bullous Pyoderma Gangrenosum With Subungual Involvement Associated With Ulcerative Colitis. ( 27893467 )
2016
28
Unusual cause of breast wound: postoperative pyoderma gangrenosum. ( 27444684 )
2016
29
High-dose ustekinumab for the treatment of severe, recalcitrant pyoderma gangrenosum. ( 27502191 )
2016
30
Pyoderma gangrenosum: A clinico-epidemiological study. ( 27549869 )
2016
31
Drug-induced pyoderma gangrenosum: a model to understand the pathogenesis of pyoderma gangrenosum. ( 27864925 )
2016
32
Hyperbaric Oxygen Therapy for the Adjunctive Treatment of Pyoderma Gangrenosum: A Case Report. ( 27192719 )
2016
33
Successful treatment of large refractory pyoderma gangrenosum-like presentation of granulomatosis with polyangiitis with rituximab. ( 27126548 )
2016
34
Concomitant Pyoderma Gangrenosum and Erythema Nodosum in a Patient With Ulcerative Colitis. ( 27474109 )
2016
35
Ocular pyoderma gangrenosum: A systematic review. ( 27836332 )
2016
36
Pyoderma Gangrenosum-A New Manifestation of Wilson Disease? ( 27194899 )
2016
37
Pyoderma Gangrenosum in the Urologist Clinic. ( 27867335 )
2016
38
The diagnosis you wish you had never operated on: Pyoderma gangrenosum misdiagnosed as necrotizing fasciitis-a case report. ( 27875063 )
2016
39
Pyoderma gangrenosum associated with alitretinoin therapy. ( 27051854 )
2016
40
Cutaneous malakoplakia masquerading as pyoderma gangrenosum. ( 27524799 )
2016
41
Pyoderma gangrenosum with spleen involvement. Review of the literature and case report. ( 27900062 )
2016
42
Double Jeopardy: Autoimmune Myelofibrosis with Pyoderma Gangrenosum. ( 27401951 )
2016
43
Extensive Pyoderma Gangrenosum Associated With Granulomatosis With Polyangiitis With Both Responsive to Rituximab. ( 27660945 )
2016
44
Multiple Pyoderma Gangrenosum Ulcers Associated with Cocaine Abuse. ( 28003907 )
2016
45
Pyogenic Arthritis, Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis (PA-PASH) Syndrome: An Atypical Presentation of a Rare Syndrome. ( 27530224 )
2016
46
Treatment of Pyoderma Gangrenosum - Retrospective Multicenter Analysis of 121 Patients. ( 27060666 )
2016
47
Clinical outcomes and response of patients applying topical therapy for pyoderma gangrenosum: A prospective cohort study. ( 27502313 )
2016
48
Postsurgical pyoderma gangrenosum successfully treated with cyclosporine. ( 27852992 )
2016
49
Pyoderma gangrenosum in a caesarean wound following caesarean section with late occurrence of pyoderma gangrenosum of the breast. ( 27435999 )
2016
50
Total hip arthroplasty for femoral neck fracture with pyoderma gangrenosum patient: A case report. ( 28012329 )
2016

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 21)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
13.72 CXCL8 IL1R1 ITGB2 MEFV NLRP3 NOD2
2
Show member pathways
13.11 CXCL8 IL1R1 ITGB2 NOD2 TNF
3
Show member pathways
12.04 CXCL8 IL1R1 TNF
4 12.02 ITGB2 NOD2 TNF
5
Show member pathways
12 CXCL8 IL1R1 MEFV NLRP3 NOD2 PSTPIP1
6
Show member pathways
11.94 CXCL8 IL1R1 TNF
7 11.77 CXCL8 IL1R1 TNF
8 11.73 CXCL8 ITGB2 TNF
9 11.63 CXCL8 IL1R1 TNF
10
Show member pathways
11.62 MEFV NLRP3 NOD2 PSTPIP1
11 11.59 CXCL8 ITGB2 TNF
12 11.44 CXCL8 IL1R1 ITGB2 TNF
13 11.35 CXCL8 ITGB2 TNF
14 11.27 CXCL8 IL1R1 TNF
15 11.23 CXCL8 ITGB2 TNF
16 11.14 CXCL8 ITGB2 NLRP3 TNF
17 11.11 MEFV NLRP3 NOD2
18 10.98 IL1R1 NLRP3
19 10.96 CXCL8 TNF
20 10.92 NOD2 TNF
21 10.82 CXCL8 TNF

GO Terms for Pyoderma Gangrenosum

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

id Name GO ID Score Top Affiliating Genes
1 cell surface GO:0009986 8.92 IL1R1 ITGB2 NOD2 TNF

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

(show all 16)
id Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.83 MEFV NLRP3 NOD2 PSTPIP1
2 immune system process GO:0002376 9.8 MEFV NLRP3 NOD2 PSTPIP1
3 cellular response to lipopolysaccharide GO:0071222 9.63 CXCL8 NLRP3 TNF
4 negative regulation of inflammatory response GO:0050728 9.61 MEFV MVK NLRP3
5 regulation of inflammatory response GO:0050727 9.58 IL1R1 NLRP3 NOD2
6 receptor internalization GO:0031623 9.56 CXCL8 ITGB2
7 positive regulation of interleukin-8 production GO:0032757 9.55 NOD2 TNF
8 positive regulation of interleukin-1 beta secretion GO:0050718 9.54 NLRP3 NOD2
9 positive regulation of NIK/NF-kappaB signaling GO:1901224 9.51 NOD2 TNF
10 embryonic digestive tract development GO:0048566 9.48 CXCL8 TNF
11 defense response GO:0006952 9.46 CXCL8 NLRP3 NOD2 TNF
12 positive regulation of type 2 immune response GO:0002830 9.37 NLRP3 NOD2
13 cellular extravasation GO:0045123 9.32 ITGB2 TNF
14 detection of biotic stimulus GO:0009595 9.26 NLRP3 NOD2
15 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.26 ITGB2 NLRP3 NOD2 TNF
16 inflammatory response GO:0006954 9.1 CXCL8 ITGB2 MEFV NLRP3 PSTPIP1 TNF

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

id Name GO ID Score Top Affiliating Genes
1 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
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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