The Pathophysiology and Treatment of Pyoderma Gangrenosum-Current Options and New Perspectives

Overview

Journal Int J Mol Sci

Publisher MDPI

Specialties Biochemistry
Chemistry
Molecular Biology

Date 2024 Feb 24

PMID 38397117

Authors

Magdalena Lyko

Anna Rygula

Michal Kowalski

Julia Karska

Alina Jankowska-Konsur

Affiliations

Soon will be listed here.

Abstract

Pyoderma gangrenosum (PG) is an uncommon inflammatory dermatological disorder characterized by painful ulcers that quickly spread peripherally. The pathophysiology of PG is not fully understood; however, it is most commonly considered a disease in the spectrum of neutrophilic dermatoses. The treatment of PG remains challenging due to the lack of generally accepted therapeutic guidelines. Existing therapeutic methods focus on limiting inflammation through the use of immunosuppressive and immunomodulatory therapies. Recently, several reports have indicated the successful use of biologic drugs and small molecules administered for coexisting diseases, resulting in ulcer healing. In this review, we summarize the discoveries regarding the pathophysiology of PG and present treatment options to raise awareness and improve the management of this rare entity.

Citing Articles

Advancing Pyoderma Gangrenosum Management With a Multidisciplinary Approach.

Grace M, Estil-Las A, Arsene C Cureus. 2025; 17(1):e77879.

PMID: 39991328 PMC: 11847161. DOI: 10.7759/cureus.77879.

Paraneoplastic Dermatoses: A Clue for Underlying Malignancies.

Didona D, Rallo A, Carugno A, Paolino G J Clin Med. 2025; 14(3).

PMID: 39941684 PMC: 11818922. DOI: 10.3390/jcm14031014.

Pyoderma Gangrenosum: A Nightmare for Breast Surgery-Two Case Reports.

Akoglu G, Demiriz M, Yilmaz K Eur J Breast Health. 2025; 21(1):80-84.

PMID: 39744929 PMC: 11706121. DOI: 10.4274/ejbh.galenos.2024.2024-7-5.

[Postoperatively developing ulcerous skin lesions with associated ulcerative colitis in a patient with status after femorocrural (P3) bypass insertion for peripheral arterial occlusion disease].

Barth U, Eltokhy M, Meyer F, Langer D, Halloul Z Chirurgie (Heidelb). 2024; 96(2):145-149.

PMID: 39514002 PMC: 11785625. DOI: 10.1007/s00104-024-02190-y.

References

PERRY H . Pyoderma gangrenosum. South Med J. 1969; 62(8):899-908. DOI: 10.1097/00007611-196908000-00001. View

Pastor N, Betlloch I, Pascual J, Blanes M, Banuls J, Silvestre J . Pyoderma gangrenosum treated with anti-TNF alpha therapy (etanercept). Clin Exp Dermatol. 2005; 31(1):152-3. DOI: 10.1111/j.1365-2230.2005.01972.x. View

Nesterovitch A, Gyorfy Z, Hoffman M, Moore E, Elbuluk N, Tryniszewska B . Alteration in the gene encoding protein tyrosine phosphatase nonreceptor type 6 (PTPN6/SHP1) may contribute to neutrophilic dermatoses. Am J Pathol. 2011; 178(4):1434-41. PMC: 3078441. DOI: 10.1016/j.ajpath.2010.12.035. View

Boutet M, Bart G, Penhoat M, Amiaud J, Brulin B, Charrier C . Distinct expression of interleukin (IL)-36α, β and γ, their antagonist IL-36Ra and IL-38 in psoriasis, rheumatoid arthritis and Crohn's disease. Clin Exp Immunol. 2015; 184(2):159-73. PMC: 4837235. DOI: 10.1111/cei.12761. View

Lorenz U . SHP-1 and SHP-2 in T cells: two phosphatases functioning at many levels. Immunol Rev. 2009; 228(1):342-59. PMC: 2669678. DOI: 10.1111/j.1600-065X.2008.00760.x. View

Pappu R, Ramirez-Carrozzi V, Sambandam A . The interleukin-17 cytokine family: critical players in host defence and inflammatory diseases. Immunology. 2011; 134(1):8-16. PMC: 3173690. DOI: 10.1111/j.1365-2567.2011.03465.x. View

Kolios A, Gubeli A, Meier B, Maul J, Kundig T, Nilsson J . Clinical Disease Patterns in a Regional Swiss Cohort of 34 Pyoderma Gangrenosum Patients. Dermatology. 2017; 233(4):268-276. DOI: 10.1159/000481432. View

Ben Abdallah H, Fogh K, Bech R . Pyoderma gangrenosum and tumour necrosis factor alpha inhibitors: A semi-systematic review. Int Wound J. 2019; 16(2):511-521. PMC: 7949186. DOI: 10.1111/iwj.13067. View

Kikuchi N, Hiraiwa T, Ohashi T, Hanami Y, Satoh M, Takenoshita H . Pyoderma gangrenosum possibly triggered by adalimumab. Eur J Dermatol. 2012; 22(6):804-5. DOI: 10.1684/ejd.2012.1849. View

10.

Braun-Falco M, Kovnerystyy O, Lohse P, Ruzicka T . Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH)--a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol. 2011; 66(3):409-15. DOI: 10.1016/j.jaad.2010.12.025. View

11.

Xu A, Balgobind A, Strunk A, Garg A, Alloo A . Prevalence estimates for pyoderma gangrenosum in the United States: An age- and sex-adjusted population analysis. J Am Acad Dermatol. 2019; 83(2):425-429. DOI: 10.1016/j.jaad.2019.08.001. View

12.

Monari P, Moro R, Motolese A, Misciali C, Baraldi C, Fanti P . Epidemiology of pyoderma gangrenosum: Results from an Italian prospective multicentre study. Int Wound J. 2018; 15(6):875-879. PMC: 7949684. DOI: 10.1111/iwj.12939. View

13.

Rogge F, Pacifico M, Kang N . Treatment of pyoderma gangrenosum with the anti-TNFalpha drug - Etanercept. J Plast Reconstr Aesthet Surg. 2008; 61(4):431-3. DOI: 10.1016/j.bjps.2006.11.011. View

14.

Nasifoglu S, Heinrich B, Welzel J . Successful therapy for pyoderma gangrenosum with a Janus kinase 2 inhibitor. Br J Dermatol. 2018; 179(2):504-505. DOI: 10.1111/bjd.16468. View

15.

Smith S, Wu P, Seo J, Fernando T, Jin M, Contreras J . IL-16/miR-125a axis controls neutrophil recruitment in pristane-induced lung inflammation. JCI Insight. 2018; 3(15). PMC: 6129123. DOI: 10.1172/jci.insight.120798. View

16.

dordevic Betetto L, Tockova O, Bergant Suhodolcan A . Mucocutaneous pyoderma gangrenosum: a case report and literature review. Acta Dermatovenerol Alp Pannonica Adriat. 2022; 31(Suppl):S10-S13. View

17.

Satoh T, Mellett M, Contassot E, French L . Are neutrophilic dermatoses autoinflammatory disorders?. Br J Dermatol. 2016; 178(3):603-613. DOI: 10.1111/bjd.15105. View

18.

Marzano A, Damiani G, Ceccherini I, Berti E, Gattorno M, Cugno M . Autoinflammation in pyoderma gangrenosum and its syndromic form (pyoderma gangrenosum, acne and suppurative hidradenitis). Br J Dermatol. 2016; 176(6):1588-1598. DOI: 10.1111/bjd.15226. View

19.

Johnston A, Xing X, Wolterink L, Barnes D, Yin Z, Reingold L . IL-1 and IL-36 are dominant cytokines in generalized pustular psoriasis. J Allergy Clin Immunol. 2017; 140(1):109-120. PMC: 5494022. DOI: 10.1016/j.jaci.2016.08.056. View

20.

Borda L, Wong L, Marzano A, Ortega-Loayza A . Extracutaneous involvement of pyoderma gangrenosum. Arch Dermatol Res. 2019; 311(6):425-434. DOI: 10.1007/s00403-019-01912-1. View