Skip to main content

Advertisement

SpringerLink
Log in

Limb amputation in patients with pyoderma gangrenosum: a multi-institutional case series

  • SHORT REPORT
  • Published:
Archives of Dermatological Research Aims and scope Submit manuscript

Abstract

Introduction

Pyoderma gangrenosum (PG) can represent a diagnostic challenge, leading to missed or delayed diagnosis. With prolonged immunosuppressive therapy, the risk of infections is elevated, predisposing patients to receive anti-infective treatments and, in serious cases, amputations. Limb amputations have been reported as complication of PG misdiagnosis but can also occur as a complication of long-standing PG ulcers.

Methods

We aimed to describe the clinical characteristics of patients with PG leading to limb amputation through a multicenter retrospective case series between 2010 and 2020 including patients with PG who underwent limb amputation. We report a descriptive analysis of these patients’ clinical course and outcome.

Results

Ten patients with PG who underwent at least one limb amputation were identified. Six were male (60%). Mean age was 65 years. All patients had ulcerative PG on the lower extremities, with a mean PG ulcer duration of 30.6 months. Six patients had PG-related comorbidities such as ulcerative colitis, myelodysplasia, and inflammatory arthritis. Other significant comorbidities included diabetes mellitus (DM) (five patients), coronary artery disease (five patients), and chronic kidney disease (two patients). The majority of patients (8/10) were correctly diagnosed with PG prior to amputation, whereas two patients were misdiagnosed with necrotizing soft tissue infections (NSTIs). All patients received intravenous antibiotics without substantial improvement. Eight patients developed sepsis and shock-like symptoms and the diagnosis of NSTIs was considered. Below-knee amputation was performed in six patients and above-knee amputation in four. Four patients had amputation performed twice because of recurrent NSTIs.

Conclusion This multicenter case series sheds light on practice gaps for physician assessing patients with PG, in that limb amputation may result from PG misdiagnosis or complications thereof. Elderly patients (above 65 years) with coexisting lower extremity PG, DM, and/or chronic cardiac or renal disease should be managed with particular care toward preventing infection/NSTIs to prevent further complications such as limb amputations.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Data availability

All data generated or analysed during this study are included in this published article and its supplementary information files.

References

  1. Barańska-Rybak W, Kakol M, Naesstrom M, Komorowska O, Sokołowska-Wojdyło M, Roszkiewicz J (2011) A retrospective study of 12 cases of pyoderma gangrenosum: why we should avoid surgical intervention and what therapy to apply. Am Surg 77(12):1644–1649

    Article  PubMed  Google Scholar 

  2. Ben Abdallah H, Bech R, Fogh K, Olesen AB, Vestergaard C (2021) Comorbidities, mortality and survival in patients with pyoderma gangrenosum: a Danish nationwide registry-nested case-control study. Br J Dermatol. https://doi.org/10.1111/bjd.20474

    Article  PubMed  Google Scholar 

  3. Bennett ML, Jackson JM, Jorizzo JL, Fleischer AB Jr, White WL, Callen JP (2000) Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions. Medicine (Baltimore) 79(1):37–46

    Article  CAS  PubMed  Google Scholar 

  4. Binus AM, Qureshi AA, Li VW, Winterfield LS (2011) Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br J Dermatol 165(6):1244–1250

    Article  CAS  PubMed  Google Scholar 

  5. Cirotteau P, Heron-Mermin D, Dimicoli-Salazar S, Gérard E, Leroy H, Clément L, Beylot-Barry M (2019) Pyoderma gangrenosum misdiagnosed as necrotising fasciitis or a real association between the two? J Eur Acad Dermatol Venereol 33(8):e305–e306

    Article  CAS  PubMed  Google Scholar 

  6. Gavioli EM, Casias M, Ngo L (2020) Pyoderma gangrenosum and superimposed infection: a case report. Adv Skin Wound Care 33(6):1–3

    Article  PubMed  Google Scholar 

  7. Haag C, Hansen T, Hajar T, Latour E, Keller J, Shinkai K, Ortega-Loayza AG (2021) Comparison of three diagnostic frameworks for pyoderma gangrenosum. J Invest Dermatol 141(1):59–63

    Article  CAS  PubMed  Google Scholar 

  8. Haag CK, Nutan F, Cyrus JW, Satpathy J, Shinkai K, Ortega Loayza AG (2019) Pyoderma gangrenosum misdiagnosis resulting in amputation: a review. J Trauma Acute Care Surg 86(2):307–313

    Article  PubMed  Google Scholar 

  9. Jemec GB, Konradsen L (1994) Pyoderma gangrenosum complicated by necrotizing fasciitis. Cutis 53(3):139–141

    CAS  PubMed  Google Scholar 

  10. Jockenhöfer F, Klode J, Kröger K, Roesch A, Al Ghazal P, Dissemond J (2016) Patients with pyoderma gangrenosum: analyses of the German DRG data from 2012. Int Wound J 13(5):951–956

    Article  PubMed  Google Scholar 

  11. Nawijn F, Smeeing DPJ, Houwert RM, Leenen LPH, Hietbrink F (2020) Time is of the essence when treating necrotizing soft tissue infections: a systematic review and meta-analysis. World J Emerg Surg 15:4

    Article  PubMed  PubMed Central  Google Scholar 

  12. Sanchez IM, Lowenstein S, Johnson KA, Babik J, Haag C, Keller JJ, Ortega-Loayza AG, Cohen J, McCalmont TH, Demer AM et al (2019) Clinical features of neutrophilic dermatosis variants resembling necrotizing fasciitis. JAMA Dermatol 155(1):79–84

    Article  PubMed  Google Scholar 

  13. Shaigany S, Wong PW, Caplan A, Kim RH, Femia A (2021) Diagnostic work-up and treatment in patients with pyoderma gangrenosum: retrospective analysis of US insurance claims-based data. Arch Dermatol Res. https://doi.org/10.1007/s00403-021-02278-z

    Article  PubMed  Google Scholar 

  14. Weenig RH, Davis MD, Dahl PR, Su WP (2002) Skin ulcers misdiagnosed as pyoderma gangrenosum. N Engl J Med 347(18):1412–1418

    Article  PubMed  Google Scholar 

  15. Xia FD, Liu K, Lockwood S, Butler D, Tsiaras WG, Joyce C, Mostaghimi A (2018) Risk of developing pyoderma gangrenosum after procedures in patients with a known history of pyoderma gangrenosum-A retrospective analysis. J Am Acad Dermatol 78(2):310-314.e311

    Article  PubMed  Google Scholar 

Download references

Funding

There is no funding source.

Author information

Authors and Affiliations

  1. Department of Dermatology, Oregon Health and Science University, 3303 SW Bond Ave, CH16D, Portland, OR, 97239, USA

    Hadir Shakshouk & Alex G. Ortega‐Loayza

  2. Department of Dermatology, Mayo Clinic, Rochester, MN, USA

    Julia S. Lehman

  3. Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA, USA

    Bicong Wu & Michi M. Shinohara

  4. Division of Dermatology, Department of Medicine, University of Washington, Seattle, WA, USA

    Michi M. Shinohara

  5. West Sound Dermatology, Poulsbo, WA, USA

    Eric Xia

  6. Department of Dermatology, Brigham and Women’s Hospital, Boston, MA, USA

    Eric Xia & Arash Mostaghimi

  7. Boston University School of Medicine, Boston, MA, USA

    Eric Xia

Authors
  1. Hadir Shakshouk
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Julia S. Lehman
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Bicong Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Michi M. Shinohara
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Simone Ince
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Eric Xia
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Arash Mostaghimi
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Alex G. Ortega‐Loayza
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

A.O. designed the study. H.S., B.W., S.I. and E.X. collected the data. H.S. and A.O. wrote the main manuscript. J.L., M.S. and A.M. reviewed the manuscript.

Corresponding author

Correspondence to Alex G. Ortega‐Loayza.

Ethics declarations

Conflict of interest

None declared.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Supplementary Information

Below is the link to the electronic supplementary material.

Supplementary file1 (DOCX 17 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Shakshouk, H., Lehman, J.S., Wu, B. et al. Limb amputation in patients with pyoderma gangrenosum: a multi-institutional case series. Arch Dermatol Res 315, 1443–1448 (2023). https://doi.org/10.1007/s00403-022-02484-3

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00403-022-02484-3

Keywords

Search

Navigation