Details of Disease

General Information of Disease (ID: DIS8QVTT)

Disease Name	Pyoderma gangrenosum
Disease Class	EB21: Pyoderma gangrenosum
Definition	Pyoderma gangrenosum (PG) is a primarily sterile inflammatory neutrophilic dermatosis characterized by recurrent cutaneous ulcerations with a mucopurulent or hemorrhagic exudate.
Disease Hierarchy	DISCMCGL: Autoinflammatory syndrome DISZ6M7Y: Pyoderma DIS8QVTT: Pyoderma gangrenosum
ICD Code	ICD-11 ICD-11: EB21 Expand ICD-11 'EB21 Expand ICD-10 'L00-L08; 'L08.0; 'L80-L99; 'L88
Disease Identifiers	MONDO ID MONDO_0018824 MESH ID D017511 UMLS CUI C0085652 MedGen ID 43224 HPO ID HP:0025452 Orphanet ID 48104 SNOMED CT ID 74578003

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 3 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Adalimumab	DMQMV1B	Approved	Antibody	[1]
Clofazimine	DMEBOFW	Approved	Small molecular drug	[2]
Etrasimod	DMT0A3Y	Approved	NA	[3]
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This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
MABp1	DM8D13S	Phase 3	NA	[3]
Sivelestat	DM6BZCV	Phase 3	Small molecular drug	[3]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 2 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
NLRP3	TT4EN8X	Strong	Biomarker	[4]
NOD2	TTYPUHA	Strong	Biomarker	[4]
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This Disease Is Related to 10 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
DERL1	OTJUS74N	Limited	Genetic Variation	[5]
GLI3	OTKDOE94	Limited	Biomarker	[4]
PAPPA	OTTTG9PG	Limited	Biomarker	[4]
SYN3	OTSGYNA5	Limited	Genetic Variation	[6]
LAD1	OT6YGTVX	Strong	Biomarker	[7]
MAP6	OTPUI00F	Strong	Genetic Variation	[8]
NELFCD	OTOLTZPU	Strong	Altered Expression	[9]
RAG1	OTV131E4	Strong	Genetic Variation	[10]
TRAF3IP2	OTLLZERL	Strong	Genetic Variation	[11]
MEFV	OTRJ6S6K	Definitive	Genetic Variation	[12]
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⏷ Show the Full List of 10 DOT(s)

References

1	An update on adalimumab for pyoderma gangrenosum. Drugs Today (Barc). 2021 Sep;57(9):535-542.
2	Clofazimine FDA Label
3	Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
4	Pyoderma gangrenosum and its syndromic forms: evidence for a link with autoinflammation.Br J Dermatol. 2016 Nov;175(5):882-891. doi: 10.1111/bjd.14691. Epub 2016 Aug 23.
5	Association of pyoderma gangrenosum and sterile osteomyelitis in a patient having myelodysplastic syndrome with der(1;7)(q10;q10).Eur J Haematol. 2004 Feb;72(2):149-53. doi: 10.1046/j.0902-4441.2003.00191.x.
6	Clinical, serologic, and genetic factors associated with pyoderma gangrenosum and erythema nodosum in inflammatory bowel disease patients.Inflamm Bowel Dis. 2014 Mar;20(3):525-33. doi: 10.1097/01.MIB.0000442011.60285.68.
7	Leukocyte adhesion deficiency-I with a novel intronic mutation presenting with pyoderma gangrenosum- like lesions.J Clin Immunol. 2015 May;35(4):431-4. doi: 10.1007/s10875-015-0155-3. Epub 2015 Apr 16.
8	Ciclosporin compared with prednisolone therapy for patients with pyoderma gangrenosum: cost-effectiveness analysis of the STOP GAP trial.Br J Dermatol. 2017 Dec;177(6):1527-1536. doi: 10.1111/bjd.15561. Epub 2017 May 31.
9	T helper type 1-related molecules as well as interleukin-15 are hyperexpressed in the skin lesions of patients with pyoderma gangrenosum.Clin Exp Immunol. 2017 Sep;189(3):383-391. doi: 10.1111/cei.12989. Epub 2017 Jun 23.
10	Three faces of recombination activating gene 1 (RAG1) mutations.Acta Microbiol Immunol Hung. 2015 Dec;62(4):393-401. doi: 10.1556/030.62.2015.4.4.
11	TRAF3IP2 gene is associated with cutaneous extraintestinal manifestations in inflammatory bowel disease.J Crohns Colitis. 2013 Feb;7(1):44-52. doi: 10.1016/j.crohns.2012.02.020. Epub 2012 Mar 24.
12	Autoinflammation in pyoderma gangrenosum and its syndromic form (pyoderma gangrenosum, acne and suppurative hidradenitis).Br J Dermatol. 2017 Jun;176(6):1588-1598. doi: 10.1111/bjd.15226. Epub 2017 Apr 16.