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Dermatology

Fever, Hypotension, and a Worsening Necrotic Wound

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.

1 Credit CME

JAMA

Published Online: March 21, 2022

JAMA Clinical Challenge

Article Information and Disclosures

Danielle M. Peterson, MD¹;

William E. Damsky, MD, PhD^1,2;

Matthew D. Vesely, MD, PhD¹

Author Affiliations

¹Department of Dermatology, Yale School of Medicine, New Haven, Connecticut
²Department of Pathology, Yale School of Medicine, New Haven, Connecticut

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On the day of giving birth via a normal vaginal delivery, a healthy woman in her 20s developed painful swelling on her right thigh, at the site of a methergine injection administered 1 day prior. Despite 3 days of treatment with an oral antibiotic (cefalexin), her thigh pain and swelling did not improve, and she was readmitted to the hospital and intravenous clindamycin was started. Wound cultures and blood cultures obtained during incision and drainage performed on hospital day 1 were negative for bacterial, mycobacterial, and fungal organisms. The following day, her temperature was 39.4 °C (102.9 °F), blood pressure was 86/42 mm Hg, and heart rate was 131/min. She was transferred to the intensive care unit for presumed septic shock, and her antibiotics were changed to vancomycin and meropenem. Surgical debridement of the right thigh was performed on hospital day 3.

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Necrotizing pyoderma gangrenosum

D. Start intravenous glucocorticoids

The key to the correct diagnosis of necrotizing pyoderma gangrenosum in this case was the presence of a dusky ulcer with a violaceous border, histopathologic findings of a dense pan-dermal neutrophilic infiltrate (Figure, right), and absence of an infectious organism on multiple blood and tissue cultures. Because pyoderma gangrenosum is an inflammatory condition, additional antifungal therapy (choice A) is not effective, and additional surgical debridement (choice B) could worsen the condition because of pathergy. Hyperbaric oxygen therapy (choice C) may be used for wound healing but would not resolve the inflammation associated with pyoderma gangrenosum.

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Article Information

Corresponding Author: Matthew D. Vesely, MD, PhD, Department of Dermatology, Yale School of Medicine, 333 Cedar St, PO Box 208059, New Haven, CT 06510 (matthew.vesely@yale.edu).

Published Online: March 21, 2022. doi:10.1001/jama.2022.2806

Conflict of Interest Disclosures: Dr Vesely reported that his spouse is an employee of Regeneron Pharmaceuticals. Dr Damsky reported serving as a consultant for Pfizer, Eli Lilly, and TWi Biotechnology; receiving research funding from Pfizer; and receiving licensing fees from MilliporeSigma. No other disclosures were reported.

Funding/Support: Dr Vesely is supported by a Dermatology Foundation Career Development Award, the Melanoma Research Alliance, and the National Center for Advancing Translational Sciences (KL2 TR001862). Dr Damsky is supported by the National Institute of Allergy and Infectious Diseases (K08AI159229).

Role of the Funder/Sponsor: The funders had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Additional Contributions: We thank the patient for providing permission to share her information.

References

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Nelson CA , Stephen S , Ashchyan HJ , James WD , Micheletti RG , Rosenbach M . Neutrophilic dermatoses: pathogenesis, Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease. J Am Acad Dermatol. 2018;79(6):987-1006. doi:10.1016/j.jaad.2017.11.064 PubMed Google Scholar Crossref

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. 2020;83(2):425-429. doi:10.1016/j.jaad.2019.08.001 PubMed Google Scholar Crossref

Sanchez IM , Lowenstein S , Johnson KA , et al. Clinical features of neutrophilic dermatosis variants resembling necrotizing fasciitis. JAMA Dermatol. 2019;155(1):79-84. doi:10.1001/jamadermatol.2018.3890 PubMed Google Scholar Crossref

Tolkachjov SN , Fahy AS , Cerci FB , Wetter DA , Cha SS , Camilleri MJ . Postoperative pyoderma gangrenosum: a clinical review of published cases. Mayo Clin Proc. 2016;91(9):1267-1279. doi:10.1016/j.mayocp.2016.05.001 PubMed Google Scholar Crossref

Ashchyan HJ , Butler DC , Nelson CA , et al. The association of age with clinical presentation and comorbidities of pyoderma gangrenosum. JAMA Dermatol. 2018;154(4):409-413. doi:10.1001/jamadermatol.2017.5978 PubMed Google Scholar Crossref

Kroshinsky D , Alloo A , Rothschild B , et al. Necrotizing Sweet syndrome: a new variant of neutrophilic dermatosis mimicking necrotizing fasciitis. J Am Acad Dermatol. 2012;67(5):945-954. doi:10.1016/j.jaad.2012.02.024 PubMed Google Scholar Crossref

Maverakis E , Ma C , Shinkai K , et al. Diagnostic criteria of ulcerative pyoderma gangrenosum: a Delphi consensus of international experts. JAMA Dermatol. 2018;154(4):461-466. doi:10.1001/jamadermatol.2017.5980 PubMed Google Scholar Crossref

Jockenhöfer F , Wollina U , Salva KA , Benson S , Dissemond J . The PARACELSUS score: a novel diagnostic tool for pyoderma gangrenosum. Br J Dermatol. 2019;180(3):615-620. doi:10.1111/bjd.16401 PubMed Google Scholar Crossref

Ormerod AD , Thomas KS , Craig FE , et al; UK Dermatology Clinical Trials Network’s STOP GAP Team. Comparison of the two most commonly used treatments for pyoderma gangrenosum: results of the STOP GAP randomised controlled trial. BMJ. 2015;350:h2958. doi:10.1136/bmj.h2958 PubMed Google Scholar Crossref

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.