January 2019

Necrotizing Neutrophilic Dermatitis, an Often-Misdiagnosed Entity With Potentially Severe Consequences

Jeffrey P. Callen, MD¹

Author Affiliations

JAMA Dermatol. 2019;155(1):17-18. doi:10.1001/jamadermatol.2018.3788

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I first became aware of the diagnosis of pyoderma gangrenosum (PG) early in my dermatology residency when I encountered a patient diagnosed with ulcerative colitis who presented with fever and hundreds of vesiculopustular lesions on her face, trunk, and extremities, several of which developed into typical lesions of classic PG.¹Within weeks of seeing that patient, I cared for another patient with a known diagnosis of agnogenic myeloid metaplasia with an atypical, superficial variant of PG that has been described more frequently on the upper extremities and face in association with a myeloid preleukemic process.² This rare ulcerative disease, first described in 1930 by Brunsting et al,³ was something I heard little of during medical school or my medicine residency. However, within the initial year of my dermatology residency I had become involved with multiple cases of PG and I was hooked on investigating the literature, writing about my experiences with misdiagnoses and management of patients beyond the use of systemic corticosteroids. From 1977, when my first publication on this entity appeared in this journal,² until today, I have been an author or coauthor on 28 publications about PG. The most recent publication is part of a Delphi project to develop improved diagnostic criteria⁴ that went beyond the statement by Perry in 1969 that “the diagnosis of a skin lesion as pyoderma gangrenosum (PG) is dependent entirely upon the clinical evaluation of the lesion. Unfortunately, there are no characteristic histopathologic changes or abnormalities on laboratory tests.”^5(p899)