Pyoderma Gangrenosum malady

NIH Rare Diseases: 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.³⁰

MalaCards: Pyoderma Gangrenosum, also known as pyoderma gangrenosum (disorder), is related to pyogenic arthritis, pyoderma gangrenosum and acne and arthritis. An important gene associated with Pyoderma Gangrenosum is PSTPIP1 (proline-serine-threonine phosphatase interacting protein 1), and among its related pathways are Immune response_Bacterial infections in normal airways and Immune System. The drugs clofazimine and sulfapyridine and the compounds cyclophosphamide and epinephrine have been mentioned in the context of this disorder. Affiliated tissues include skin and monocytes, and related mouse phenotypes are hematopoietic system and skeleton.

Wikipedia: Pyoderma gangrenosum is a condition that causes tissue to become necrotic, causing deep ulcers that...⁴⁴ more...

Aliases & Descriptions:

pyoderma gangrenosum 6 30 8 32 43 pyoderma gangrenosum (disorder) 6

Diseases related to pyoderma gangrenosum by text searches and GeneDecks gene sharing:

(show top 50)    (show all 476)

id	Related Disease	Score	Top Affiliating Genes
1	pyogenic arthritis, pyoderma gangrenosum and acne	34.2	MEFV, PTPN18, PSTPIP1
2	arthritis	30.9	IL6, IL8, TNF
3	acne	29.8	MMP1, IL1R1, IL8, IFNA2, TNF, NOD2
4	antiphospholipid syndrome	29.2	CD40LG, APOH, F3, TNF
5	myelodysplastic syndrome	28.7	CD40LG, CD55, MEFV, IL6, IL8, IFNA2
6	acquired immunodeficiency syndrome	27.9	CD40LG, IL6, APOH, IFNA2, F3, TNF
7	immunodeficiency	27.6	CD40LG, CD55, ITGB2, IL6, APOH, TNF
8	inflammatory bowel disease	27.1	CD55, ITGB2, MMP1, IL1R1, IL6, IL8
9	wegener's granulomatosis	26.6	ITGB2, IL6, IL8, APOH, F3, TNF
10	pyoderma	26.3	MVK, CD40LG, CD55, ITGB2, MMP1, MMP10
11	ulcerative colitis	26.0	CD55, ITGB2, MMP1, IL1R1, IL6, IL8
12	hodgkin's lymphoma	24.6	CD40LG, CD55, ITGB2, IL6, IL8, APOH
13	psoriasis	24.2	SERPINB13, CD40LG, CD55, ITGB2, MMP1, IL1R1
14	thrombosis	23.7	CD40LG, CD55, ITGB2, MMP1, MEFV, IL1R1
15	mevalonic aciduria	13.5	MVK, IDS, NLRP3
16	cryopyrin-associated periodic syndrome	13.5	IL1R1, NLRP3
17	familial cold autoinflammatory syndrome	13.5	MEFV, IL1R1, NLRP3
18	colchicine resistance	13.5	MEFV, IL1R1
19	cinca syndrome	13.4	IL1R1, NLRP3
20	discoid lupus erythematosus of eyelid	13.4	APOH, IFNA2
21	chronic infantile neurological cutaneous articular syndrome	13.4	MVK, MEFV, IL1R1, NLRP3
22	degos disease	13.4	APOH, IFNA2
23	granulomatous dermatitis	13.3	TNF, NOD2
24	periodic fever, familial, autosomal dominant	13.3	MVK, MEFV, TNF
25	familial mediterranean fever	13.3	MEFV, TNF, NLRP3
26	erythema nodosum, idiopathic	13.2	TNF, NOD2
27	mononeuropathy	13.2	APOH, IFNA2, PRTN3
28	transverse myelitis	13.2	CD40LG, APOH
29	sapho syndrome	13.1	TNF, NOD2, PSTPIP2
30	peripheral vertigo	13.1	APOH, F3
31	vulvar vestibulitis syndrome	13.1	IL1R1, TNF, NLRP3
32	panuveitis	13.1	IFNA2, TNF
33	acquired von willebrand syndrome	13.1	CD40LG, F3
34	purpura fulminans	13.0	APOH, F3, CSF3
35	erysipelas	13.0	CD40LG, MEFV, TNF
36	adult-onset still's disease	13.0	IL1R1, IL6
37	pulmonary plasma cell granuloma	13.0	TNF, NOD2, PRTN3
38	catastrophic antiphospholipid syndrome	13.0	CD40LG, APOH, F3
39	portal vein thrombosis	12.9	APOH, IFNA2, F3
40	retinal vasculitis	12.9	APOH, IFNA2, TNF
41	splenomegaly	12.9	ITGB2, IFNA2, CSF3
42	hematuria	12.9	CD40LG, F3, PRTN3
43	protein c deficiency	12.9	APOH, F3, CSF3
44	sycp3-related pregnancy loss, susceptibility to	12.9	CD40LG, APOH, F3
45	heparin-induced thrombocytopenia	12.9	CD40LG, APOH, F3
46	hyper-igd syndrome	12.9	MVK, TNF
47	cellulitis	12.9	IL1R1, TNF, CSF3
48	blau syndrome	12.9	MVK, MEFV, TNF, NOD2, NLRP3, PSTPIP1
49	acute graft versus host disease	12.8	IL1R1, TNF, NOD2, CSF3
50	hepatitis d	12.8	CD40LG, IFNA2, TNF

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 clofazimine, sulfapyridine

MalaCards organs/tissues related to pyoderma gangrenosum:

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MGI Mouse Phenotypes related to pyoderma gangrenosum:

²⁵

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	hematopoietic system phenotype	MP:0005397	9.5	CSF3, PSTPIP2, NOD2, MEFV
2	skeleton phenotype	MP:0005390	8.3	NOD2, IDS, IL6, IL1R1, MEFV, ITGB2
3	integument phenotype	MP:0010771	8.1	CSF3, PSTPIP2, NLRP3, F3, IDS, IL1R1
4	immune system phenotype	MP:0005387	7.7	MMP10, PRTN3, PSTPIP1, PSTPIP2, NOD2, TNF

Articles related to pyoderma gangrenosum:

(show all 23)

id	Title	Authors	Year	Affiliating Genes
1	Mutations in the PSTPIP1 gene and aberrant splicing v ariants in patients with pyoderma gangrenosum. (21790734)	Nesterovitch A.B.... Glant T.T.	2011	PSTPIP1
2	Failure of anakinra treatment of pyoderma gangrenosum in an IBD patient and relevance to the PSTPIP1 gene. (21438098)	Lin Z.... Koltun W.A.	2011	PSTPIP1
3	Targeted treatment of pyoderma gangrenosum in PAPA (p yogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra. (19673875)	Brenner M.... Herzer P.	2009	IL1R1
4	Reduction of interleukin-6, interleukin-8, and anti-p hosphatidylserine-prothrombin complex antibody by granulocyte and monocyte adso rption apheresis in a patient with pyoderma gangrenosum and ulcerative colitis. (19727102)	Kawakami T.... Soma Y.	2009	IL6, IL8
5	Pyogenic arthritis, pyoderma gangrenosum, and acne sy ndrome (PAPA syndrome): report of a sporadic case without an identifiable mutat ion in the CD2BP1 gene. (19700023)	Hong J.B.... Chiu H.C.	2009	PSTPIP1
6	Wegener's granulomatosis presenting as pyoderma gangrenosum. (18076660)	Boudny C.... Simon D.	2008	PRTN3
7	Pyoderma gangrenosum and exacerbation of psoriasis re sulting from pegylated interferon alpha and ribavirin treatment of chronic hepa titis C. (17700270)	Yurci A.... Yucesoy M.	2007	IFNA2
8	Expression of MMP-9, MMP-10 and TNF-alpha and lack of epithelial MMP-1 and MMP-26 characterize pyoderma gangrenosum. (18001410)	Bister V.... Saarialho-Kere U.	2007	MMP1, MMP10, MMP26
9	The role of anti-tumor necrosis factor-alpha therapy in Pyoderma gangrenosum associated with inflammatory bowel disease. (17428111)	ReguiaA^ Z.... Grange F.	2007	TNF
10	Successful treatment of myelodysplastic syndrome-indu ced pyoderma gangrenosum. (17179573)	Koca E.... Ozcebe O.I.	2006	IFNA2
11	Pyoderma gangrenosum complicating pegylated granulocyte colony-stimulating factor in Hodgkin lymphoma. (16371028)	Miall F.M.... Dyer M.J.	2006	CSF3
12	Pyoderma gangrenosum related to a new granulocyte col ony-stimulating factor. (16603845)	White L.E.... Medenica M.M.	2006	CSF3
13	Dramatic improvement of pyoderma gangrenosum with infliximab in a patient with PAPA syndrome. (15916580)	Stichweh D.S.... Pascual V.	2005	PSTPIP1
14	Treatment of pyoderma gangrenosum with etanercept. (15303791)	McGowan J.W.... Lynn A.	2004	TNF
15	CD2BP1 and CARD15 mutations are not associated with pyoderma gangrenosum in patients with inflammatory bowel disease. (15102098)	Newman B.... Siminovitch K.A.	2004	NOD2, PSTPIP1
16	Pyoderma gangrenosum associated with paroxysmal nocturnal hemoglobulinuria and monoclonal gammopathy. (11890301)	Matsubara K.... Mizutani H.	2002	CD55
17	Disseminated histoplasmosis presenting as pyoderma gangrenosum-like lesions in a patient with acquired immunodeficiency syndrome. (11703524)	Laochumroonvorapong P.... Levy H.	2001	CD40LG
18	Pyoderma gangrenosum following cytosine arabinoside, aclarubicin and granulocyte colony-stimulating factor combination therapy in myelodysplastic syndrome. (9617871)	Takagi S.... Matsuoka T.	1998	CSF3
19	Pyoderma gangrenosum in a child with congenital partial deficiency of leucocyte adherence glycoproteins. (9990374)	Bedlow A.J.... Marsden R.A.	1998	ITGB2
20	Pyoderma gangrenosum and extensive caval thrombosis associated with the antiphospholipid syndrome--a case report. (9482516)	Chacek S.... QuiA+ones-Galvan A.	1998	F3, APOH
21	Bullous pyoderma gangrenosum in a patient with myelodysplastic syndrome during granulocyte colony-stimulating factor therapy. (9389372)	Lewerin C.... Swolin B.	1997	CSF3
22	Fatal pyoderma gangrenosum in association with C7 deficiency. (1517503)	Friduss S.R.... Fivenson D.P.	1992	C7
23	Bullous pyoderma gangrenosum after granulocyte colony-stimulating factor treatment. (1712666)	Ross H.J.... Figlin R.A.	1991	CSF3

Genes related to pyoderma gangrenosum according to GeneCards:

(show all 28)

id	Symbol	Description	Score	GeneCards Section Context
1	PSTPIP1	proline-serine-threonine phosphatase interacting protein 1	11.1	Publications
2	MVK	mevalonate kinase	11.0
3	MEFV	Mediterranean fever	11.0
4	NLRP3	NLR family, pyrin domain containing 3	11.0
5	NOD2	nucleotide-binding oligomerization domain containing 2	11.0	Publications
6	MESDC1	mesoderm development candidate 1	11.0
7	MMP26	matrix metallopeptidase 26	11.0	Publications
8	MESDC2	mesoderm development candidate 2	11.0
9	SERPINB13	serpin peptidase inhibitor, clade B (ovalbumin), member 13	11.0
10	PTPN18	protein tyrosine phosphatase, non-receptor type 18 (brain-derived)	11.0
11	MMP10	matrix metallopeptidase 10 (stromelysin 2)	11.0	Publications
12	IDS	iduronate 2-sulfatase	11.0
13	GUCA1A	guanylate cyclase activator 1A (retina)	11.0
14	C7	complement component 7	11.0	Publications
15	PRTN3	proteinase 3	11.0	Publications
16	PSTPIP2	proline-serine-threonine phosphatase interacting protein 2	11.0
17	IL1R1	interleukin 1 receptor, type I	11.0	Publications
18	ITGB2	integrin, beta 2 (complement component 3 receptor 3 and 4 subunit)	11.0	Publications
19	APOH	apolipoprotein H (beta-2-glycoprotein I)	11.0	Publications
20	CSF3	colony stimulating factor 3 (granulocyte)	11.0	Publications
21	IFNA2	interferon, alpha 2	11.0	Publications
22	CD40LG	CD40 ligand	11.0	Publications
23	F3	coagulation factor III (thromboplastin, tissue factor)	11.0	Publications
24	MMP1	matrix metallopeptidase 1 (interstitial collagenase)	11.0	Publications
25	CD55	CD55 molecule, decay accelerating factor for complement (Cromer blood group)	11.0	Publications
26	TNF	tumor necrosis factor	11.0	Publications
27	IL6	interleukin 6 (interferon, beta 2)	11.0	Publications
28	IL8	interleukin 8	11.0	Publications

Pathways related to pyoderma gangrenosum according to GeneDecks:

(show all 35)

id	Pathway	Score	Top Affiliating Genes
1	Immune response_Bacterial infections in normal airways41	10.3	IL1R1
2	Immune System38	10.1	PSTPIP1, NLRP3, IL1R1, MEFV
3	Inhibition of Matrix Metalloproteinases36	9.9	MMP1, MMP10, MMP26
4	Immune response_Alternative complement pathway41	9.8	C7, ITGB2, CD55
5	Immune response Alternative complement pathway10	9.8	C7, ITGB2, CD55
6	Immune response_Lectin induced complement pathway41	9.7	C7, ITGB2, CD55
7	Immune response Lectin Induced complement pathway10	9.7	C7, ITGB2, CD55
8	Immune response Classic complement pathway10	9.3	C7, ITGB2, CD55
9	Immune response_Classical complement pathway41	9.1	CD55, ITGB2, C7
10	Cytokine production by Th17 cells in CF41	9.0	CSF3, IL8, IL6
11	Akt Signaling36	9.0	ITGB2, IL6, TNF
12	Cytokine production by Th17 cells in CF (Mouse model)41	8.8	IL6, IL8, CSF3
13	Immune response MIF in innate immunity response10	8.7	IL6, IL8, TNF
14	Immune response_MIF in innate immunity response41	8.7	IL6, IL8, TNF
15	Bacterial infections in CF airways41	8.6	IL1R1, IL6, IL8, TNF
16	Tuberculosis20	8.6	ITGB2, IL6, IFNA2, TNF, NOD2
17	Cytokine Network36	8.5	TNF, IFNA2, IL8, IL6
18	Toll-like receptor signaling pathway20	8.5	IL6, IL8, IFNA2, TNF
19	Pertussis20	8.5	NLRP3, TNF, IL8, IL6, ITGB2
20	MIF Mediated Glucocorticoid Regulation36	8.4	CSF3, TNF, IL8, IL6
21	Immune response_MIF-mediated glucocorticoid regulation41	8.4	TNF, IL8, IL6, MMP1
22	Immune response MIF-mediated glucocorticoid regulation10	8.4	TNF, IL8, IL6, MMP1
23	MIF Regulation of Innate Immune Cells36	8.4	CSF3, TNF, IL8, IL6
24	Influenza A20	8.4	NLRP3, TNF, IFNA2, IL8, IL6
25	NOD-like receptor signaling pathway20	8.3	MEFV, PSTPIP1, NLRP3, NOD2, TNF, IL8
26	Hematopoietic cell lineage20	8.3	CSF3, TNF, IL6, IL1R1, CD55
27	all-trans-Retinoic Acid Signaling in Brain36	8.3	IL6, IL8, TNF, CSF3
28	STAT3 Pathway36	8.2	CSF3, TNF, IL8, IL6, IL1R1
29	Immune response IL-1 signaling pathway10	8.2	TNF, F3, IL8, IL6, IL1R1
30	Immune response_IL-1 signaling pathway41	8.2	TNF, F3, IL8, IL6, IL1R1
31	PEDF Induced Signaling36	8.2	IL6, IL8, TNF, CSF3
32	Amoebiasis20	8.2	TNF, IL8, IL6, IL1R1, ITGB2, SERPINB13
33	Rheumatoid arthritis20	8.1	ITGB2, MMP1, IL6, IL8, TNF
34	Malaria20	7.9	CSF3, TNF, IL8, IL6, ITGB2, CD40LG
35	Cytokine-cytokine receptor interaction20	7.6	CSF3, TNF, IFNA2, IL8, IL6, IL1R1

Compounds related to pyoderma gangrenosum according to GeneDecks:

(show top 50)    (show all 120)

id	Compound	Score	Top Affiliating Genes
1	cyclophosphamide32 34 9 9	13.2	APOH, ITGB2
2	epinephrine32 9 18 9	13.0	F3, MMP1
3	hydroxychloroquine32 9 9	12.0	APOH, IL1R1, CD40LG
4	hyaluronic acid32 18	10.9	PRTN3, ITGB2, MMP1
5	n-formylmethionyl-leucylphenylalanine32	9.5	ITGB2, PRTN3, CD55, CSF3
6	rituximab32 34 9 9	12.5	CD55, CD40LG, PRTN3, IL1R1
7	histamine32 18	10.2	CD55, IFNA2, CD40LG, ITGB2
8	endotoxin32	9.2	ITGB2, IL1R1, CSF3, MEFV, APOH, F3
9	aebsf32	9.0	PRTN3, TNF, IL8, F3
10	anakinra32 9 9	10.9	MVK, IL6, TNF, NLRP3, IL1R1
11	sulfasalazine32 9 9	10.8	CD40LG, IL1R1, IL8, TNF
12	okt332	8.6	CD40LG, ITGB2, TNF, IL6
13	cysteine32	8.5	IDS, IFNA2, CD55, GUCA1A, CD40LG, SERPINB13
14	leflunomide32 9 9	10.5	TNF, IL6, IL1R1, MMP1
15	imiquimod32 42 9 9	11.5	IL8, TNF, IFNA2, IL6
16	il 1032	8.5	NOD2, IL8, TNF, IL1R1, IL6
17	peptidoglycan32	8.5	NOD2, TNF, IL8, IL6, IL1R1
18	polymyxin b32	8.4	TNF, IL8, IL6, ITGB2
19	thalidomide32 42 9 9	11.4	TNF, IFNA2, IL6, ITGB2, CD40LG
20	lipoteichoic acid32	8.3	IL1R1, IL6, TNF, APOH, IL8
21	gm-csf32	8.3	TNF, CSF3, IL8, IL6
22	sb 20358032 42	9.3	IL6, MMP1, IL1R1, TNF, ITGB2
23	il-1232	8.3	IL8, CD40LG, IL6, IL1R1, TNF
24	ccl332	8.3	IL8, TNF, IL6, IL1R1, CSF3
25	fmlp32	8.2	PRTN3, TNF, ITGB2, CD55, CSF3, IL8
26	8-isoprostane32	8.2	TNF, IL6, IL8, APOH
27	simvastatin32 34 42 9 18 9	13.0	F3, MVK, TNF, IL6, ITGB2, IL8
28	leukotriene b432 42 18	10.0	ITGB2, IL1R1, IL8, TNF, IL6
29	neopterin32	7.9	TNF, CD40LG, IL6, IL1R1, IL8, IFNA2
30	pdtc32	7.9	IL6, F3, TNF, IL8, MMP1
31	methotrexate32 34 42 9 9	11.9	CSF3, NLRP3, IL1R1, MMP1, APOH, TNF
32	herbimycin a32 42	8.7	TNF, IL6, MMP1, CD40LG, IL8, ITGB2
33	infliximab32 9 9	9.7	NOD2, TNF, IFNA2, APOH, IL6, PRTN3
34	indomethacin32 9 9	9.6	ITGB2, CD40LG, MMP1, IL1R1, IL6, IFNA2
35	fibrinogen32	7.6	F3, IL1R1, APOH, IL6, ITGB2, TNF
36	etanercept32 9 9	9.6	MMP1, IL1R1, TNF, IFNA2, IL6, APOH
37	atorvastatin32 34 9 18 9	11.6	CD40LG, CD55, MMP1, TNF, IL6
38	pentoxifylline32 9 9	9.5	ITGB2, MMP1, IL1R1, IL6, F3, TNF
39	sp 60012532 42	8.3	IL6, MMP1, IL8, TNF, F3
40	prednisolone32 9 9	9.3	ITGB2, CSF3, MMP1, IL1R1, IL6, IFNA2
41	creatinine32	7.3	F3, ITGB2, MVK, CD40LG, CD55, MMP1
42	rantes32	7.2	IL6, IL8, MMP1, ITGB2, CD40LG, IL1R1
43	lactate32	7.1	MMP1, CD40LG, ITGB2, IL1R1, IL6, IL8
44	actinomycin d32	7.0	CD55, IL6, IL8, F3, TNF, IL1R1
45	cyclosporin a32 42	7.7	IL6, CD40LG, IL1R1, CD55, ITGB2, MMP1
46	retinoic acid32 42 18	8.5	IL1R1, IL6, IFNA2, F3, MMP26, PRTN3
47	dexamethasone32 42 34 9 9	10.5	ITGB2, TNF, MMP1, IL8, IL6, IL1R1
48	cycloheximide32	6.4	MMP1, IL1R1, IL6, CD55, F3, TNF
49	serine32	6.3	F3, IL8, MMP10, IL1R1, PRTN3, CD55
50	vegf32	5.5	CD40LG, CD55, ITGB2, CSF3, TNF, IL1R1

Cellular components related to pyoderma gangrenosum according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	cell surface	GO:009986	8.6	CD55, IL1R1, APOH, TNF, NOD2
2	extracellular space	GO:005615	6.3	CD40LG, CSF3, TNF, F3, IFNA2, APOH

Biological processes related to pyoderma gangrenosum according to GeneDecks:

(show all 11)

id	Name	GO ID	Score	Top Affiliating Genes
1	detection of biotic stimulus	GO:009595	10.3	NOD2, NLRP3
2	nucleotide-binding domain, leucine rich repeat containing receptor signaling pathway	GO:035872	10.0	PSTPIP1, NLRP3, NOD2, MEFV
3	negative regulation of inflammatory response	GO:050728	10.0	MVK, MEFV, NLRP3
4	collagen catabolic process	GO:030574	9.6	PRTN3, MMP26, MMP10, MMP1
5	positive regulation of B cell activation	GO:050871	9.5	IL6, NOD2
6	regulation of immunoglobulin secretion	GO:051023	9.4	CD40LG, TNF
7	positive regulation of interleukin-6 production	GO:032755	8.9	NOD2, TNF, IL6
8	positive regulation of NF-kappaB transcription factor activity	GO:051092	8.9	IL6, TNF, NOD2, NLRP3
9	negative regulation of lipid storage	GO:010888	8.7	TNF, IL6
10	innate immune response	GO:045087	8.7	PSTPIP1, C7, NLRP3, NOD2, IFNA2, IL1R1
11	inflammatory response	GO:006954	7.4	CD40LG, NLRP3, TNF, IFNA2, IL8, IL6

Molecular functions related to pyoderma gangrenosum according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	protease binding	GO:002020	9.2	IL1R1, F3, TNF
2	cytokine activity	GO:005125	7.7	CD40LG, IL6, IFNA2, TNF, CSF3