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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

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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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.2017.5980PubMedGoogle ScholarCrossref
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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.h2958PubMedGoogle ScholarCrossref
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