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Pyoderma gangrenosum

MedGen UID:
43224
Concept ID:
C0085652
Disease or Syndrome
Synonym: Pyoderma Gangrenosum
SNOMED CT: Pyoderma gangrenosum (74578003)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
Concept ID:
CN307042
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
 
HPO: HP:0025452
Monarch Initiative: MONDO:0018824
Orphanet: ORPHA48104

Definition

A deep skin ulcer with a well defined border, which is usually violet or blue. The ulcer edge is often undermined (worn and damaged) and the surrounding skin is erythematous and indurated. The ulcer often starts as a small papule or collection of papules, which break down to form small ulcers with a so called cat's paw appearance. These coalesce and the central area then undergoes necrosis to form a single ulcer. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPyoderma gangrenosum
Follow this link to review classifications for Pyoderma gangrenosum in Orphanet.

Conditions with this feature

Acute febrile neutrophilic dermatosis
MedGen UID:
43097
Concept ID:
C0085077
Disease or Syndrome
Acute febrile neutrophilic dermatosis (AFND) is an autosomal dominant autoinflammatory disorder characterized by onset of recurrent fever and dermatologic abnormalities in childhood. Laboratory studies show elevated acute-phase reactants and activation of the inflammatory response, particularly IL1B (147720). Additional more variable features may include myalgia and arthralgia (summary by Masters et al., 2016).
Lazy leukocyte syndrome
MedGen UID:
78795
Concept ID:
C0272174
Disease or Syndrome
Periodic fever, immunodeficiency, and thrombocytopenia syndrome (PFITS) is an autosomal recessive immunologic disorder with variable manifestations. Common features include early-onset recurrent respiratory infections, stomatitis, and cutaneous infections. Organisms usually include bacteria such as pneumococcus, Staphylococcus, and H. influenzae, but severe viral infections, including varicella, may also occur. Laboratory investigations may show neutropenia, neutrophilia, leukocytosis, or lymphopenia, although levels of immune cells may also be normal. Detailed studies often show impaired neutrophil chemotaxis associated with increased or abnormal F-actin levels, and impaired, normal, or even increased oxidative burst, depending on the stimulus. B- and T-cell abnormalities have also been observed. Some patients develop autoimmune manifestations, including chronic thrombocytopenia, anemia, and periodic fevers, associated with activation of the inflammasome. Early death may occur; however, hematopoietic stem cell transplantation may be curative (summary by Kuhns et al., 2016, Standing et al., 2017, and Pfajfer et al., 2018).
Pyogenic arthritis-pyoderma gangrenosum-acne syndrome
MedGen UID:
346801
Concept ID:
C1858361
Disease or Syndrome
Pyogenic sterile arthritis, pyoderma gangrenosum, and acne (PAPA) is a rare autosomal dominant autoinflammatory disease that typically presents with recurrent sterile, erosive arthritis in childhood, occurring spontaneously or after minor trauma, occasionally resulting in significant joint destruction. By puberty, joint symptoms tend to subside and cutaneous symptoms predominate, including pathergy, frequently with abscesses at the sites of injections, severe cystic acne, and recurrent nonhealing sterile ulcers, often diagnosed as pyoderma gangrenosum (summary by Demidowich et al., 2012).
Immunodeficiency, common variable, 12
MedGen UID:
906018
Concept ID:
C4225277
Disease or Syndrome
Common variable immunodeficiency-12 with autoimmunity (CVID12) is an autosomal dominant complex immunologic disorder with multisystem involvement. CVID12 is mainly a primary immunodeficiency characterized by recurrent infections and associated with hypogammaglobulinemia. Notably, about half of patients develop autoimmune features, including cytopenia, as well as generalized inflammation and lymphoproliferation manifest as lymphadenopathy or hepatosplenomegaly. A smaller percentage of affected individuals (less than 20%) develop cancer, most commonly solid tumors, including lymphoma. Age at onset and disease severity are highly variable, even within the same family. There is also incomplete penetrance, such that mutation carriers may be asymptomatic, even if they have hypogammaglobulinemia. The gene involved, NFKB1, encodes a transcription factor that regulates the expression of target genes involved in the immune system, thus defining the phenotype as a disorder of immune dysregulation (summary by Fliegauf et al., 2015; Lorenzini et al., 2020). For a general description and a discussion of genetic heterogeneity of common variable immunodeficiency, see CVID1 (607594).
Immunodeficiency 107, susceptibility to invasive staphylococcus aureus infection
MedGen UID:
1823965
Concept ID:
C5774192
Disease or Syndrome
Immunodeficiency-107 with susceptibility to invasive Staphylococcus aureus infection (IMD107) is an autosomal dominant immunologic disorder characterized most often by the development of invasive and severe life-threatening infections with S. aureus affecting the skin and/or lungs. There is incomplete penetrance (about 30%) and variable expressivity. In some patients with heterozygous OTULIN mutations, an infectious agent is not identified, suggesting that low-grade infectious or even noninfectious triggers may play a role in development of the disease. The levels and function of immune cells appear normal; the molecular defect resides in fibroblasts and possibly other nonhematopoietic barrier cells that show increased susceptibility to the detrimental effects of the S. aureus alpha-toxin (Spaan et al., 2022).

Professional guidelines

PubMed

Dissemond J, Marzano AV, Hampton PJ, Ortega-Loayza AG
Drugs 2023 Sep;83(14):1255-1267. Epub 2023 Aug 23 doi: 10.1007/s40265-023-01931-3. PMID: 37610614Free PMC Article
Garg A, Malviya N, Strunk A, Wright S, Alavi A, Alhusayen R, Alikhan A, Daveluy SD, Delorme I, Goldfarb N, Gulliver W, Hamzavi I, Jaleel T, Kimball AB, Kirby JS, Kirchhof MG, Lester J, Lev-Tov H, Lowes MA, Micheletti R, Orenstein LA, Piguet V, Sayed C, Tan J, Naik HB
J Am Acad Dermatol 2022 May;86(5):1092-1101. Epub 2021 Jan 23 doi: 10.1016/j.jaad.2021.01.059. PMID: 33493574Free PMC Article
Rogler G, Singh A, Kavanaugh A, Rubin DT
Gastroenterology 2021 Oct;161(4):1118-1132. Epub 2021 Aug 3 doi: 10.1053/j.gastro.2021.07.042. PMID: 34358489Free PMC Article

Recent clinical studies

Therapy

Tan MG, Tolkachjov SN
Dermatol Clin 2024 Apr;42(2):183-192. Epub 2023 Dec 29 doi: 10.1016/j.det.2023.12.002. PMID: 38423680
Dissemond J, Marzano AV, Hampton PJ, Ortega-Loayza AG
Drugs 2023 Sep;83(14):1255-1267. Epub 2023 Aug 23 doi: 10.1007/s40265-023-01931-3. PMID: 37610614Free PMC Article
Sander M, Sander M
CMAJ 2019 Sep 23;191(38):E1058. doi: 10.1503/cmaj.190721. PMID: 31548193Free PMC Article
Soto Vilches F, Vera-Kellet C
Med Clin (Barc) 2017 Sep 20;149(6):256-260. Epub 2017 Jun 16 doi: 10.1016/j.medcli.2017.04.013. PMID: 28629662
Pompeo MQ
Wounds 2016 Jan;28(1):7-13. PMID: 26779805

Prognosis

He R, Zhao S, Cui M, Chen Y, Ma J, Li J, Wang X
Front Immunol 2023;14:1234535. Epub 2023 Oct 26 doi: 10.3389/fimmu.2023.1234535. PMID: 37954590Free PMC Article
Barbe M, Batra A, Golding S, Hammond O, Higgins JC, O'Connor A, Vlahovic TC
Clin Podiatr Med Surg 2021 Oct;38(4):577-588. doi: 10.1016/j.cpm.2021.06.002. PMID: 34538436
Rogler G, Singh A, Kavanaugh A, Rubin DT
Gastroenterology 2021 Oct;161(4):1118-1132. Epub 2021 Aug 3 doi: 10.1053/j.gastro.2021.07.042. PMID: 34358489Free PMC Article
Greuter T, Vavricka SR
Expert Rev Gastroenterol Hepatol 2019 Apr;13(4):307-317. Epub 2019 Feb 20 doi: 10.1080/17474124.2019.1574569. PMID: 30791773
Vavricka SR, Schoepfer A, Scharl M, Lakatos PL, Navarini A, Rogler G
Inflamm Bowel Dis 2015 Aug;21(8):1982-92. doi: 10.1097/MIB.0000000000000392. PMID: 26154136Free PMC Article

Clinical prediction guides

Dissemond J, Marzano AV, Hampton PJ, Ortega-Loayza AG
Drugs 2023 Sep;83(14):1255-1267. Epub 2023 Aug 23 doi: 10.1007/s40265-023-01931-3. PMID: 37610614Free PMC Article
Barbe M, Batra A, Golding S, Hammond O, Higgins JC, O'Connor A, Vlahovic TC
Clin Podiatr Med Surg 2021 Oct;38(4):577-588. doi: 10.1016/j.cpm.2021.06.002. PMID: 34538436
Rogler G, Singh A, Kavanaugh A, Rubin DT
Gastroenterology 2021 Oct;161(4):1118-1132. Epub 2021 Aug 3 doi: 10.1053/j.gastro.2021.07.042. PMID: 34358489Free PMC Article
Greuter T, Vavricka SR
Expert Rev Gastroenterol Hepatol 2019 Apr;13(4):307-317. Epub 2019 Feb 20 doi: 10.1080/17474124.2019.1574569. PMID: 30791773
Jockenhöfer F, Wollina U, Salva KA, Benson S, Dissemond J
Br J Dermatol 2019 Mar;180(3):615-620. Epub 2018 May 6 doi: 10.1111/bjd.16401. PMID: 29388188

Recent systematic reviews

Kilic Y, Kamal S, Jaffar F, Sriranganathan D, Quraishi MN, Segal JP
Inflamm Bowel Dis 2024 Feb 1;30(2):230-239. doi: 10.1093/ibd/izad061. PMID: 37042969
Kridin K, Cohen AD, Amber KT
Am J Clin Dermatol 2018 Aug;19(4):479-487. doi: 10.1007/s40257-018-0356-7. PMID: 29721816
Bissonnette C, Kauzman A, Mainville GN
Head Neck Pathol 2017 Dec;11(4):427-441. Epub 2017 Mar 8 doi: 10.1007/s12105-017-0804-3. PMID: 28275955Free PMC Article
Kechichian E, Haber R, Mourad N, El Khoury R, Jabbour S, Tomb R
Int J Dermatol 2017 May;56(5):486-495. Epub 2017 Feb 23 doi: 10.1111/ijd.13584. PMID: 28233293
Gupta AS, Ortega-Loayza AG
J Am Acad Dermatol 2017 Mar;76(3):512-518. Epub 2016 Nov 8 doi: 10.1016/j.jaad.2016.08.049. PMID: 27836332

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