Necrotizing neutrophilic dermatosis: A diagnostic challenge with a need for multi-disciplinary recognition, a case report

Abstract

Introduction Necrotizing neutrophilic dermatoses can clinically resemble necrotizing fasciitis and therefore pose a diagnostic and therapeutic challenge. Given their similar presentations, misdiagnosis and inappropriate or delayed treatments are possible. Presentation of case We discuss the case of a woman with acute myeloid leukemia who presented with fevers, chills, cough, and a leg wound. She underwent amputation of her lower extremity after she was presumed to have necrotizing fasciitis; however, symptoms persisted. She was ultimately diagnosed with and treated for necrotizing Sweet's syndrome with notable clinical improvement. Discussion Both, necrotizing neutrophilic dermatoses and necrotizing fasciitis, grossly affect the skin and are associated with rapidly progressing systemic features including fevers, chills, leukocytosis, and elevated inflammatory markers. Recent literature in dermatology addresses these similarities and the appropriate approach to management; however, it is critical that medical and surgical subspecialties have an understanding of necrotizing neutrophilic dermatoses and their clinical presentations, diagnostic approaches, as well as therapeutic interventions. Familiarity with this entity can mitigate the risk of misdiagnosis, morbidity, and mortality. Conclusion With this report, we seek to review the features that are suggestive of and aid in the diagnosis of necrotizing neutrophilic dermatoses to help prevent significant and avoidable morbidity.

New Practical Aspects of Sweet Syndrome

“…More recently, seven additional cases of necrotizing SS have been added to the literature [44][45][46][47][48]. In all reports, the authors emphasize the need to distinguish necrotizing SS from necrotizing fasciitis, as the surgical debridement of necrotizing SS can lead to further exacerbation of SS and involvement of additional tissue.…”

“…In all reports, the authors emphasize the need to distinguish necrotizing SS from necrotizing fasciitis, as the surgical debridement of necrotizing SS can lead to further exacerbation of SS and involvement of additional tissue. Four of these recently reported cases regressed completely following steroid treatment [44,[46][47][48], two responded completely to dapsone and steroid combination therapy [44], and one patient experienced lesion regression following discontinuation of bortezomib (the putative offending agent) and steroid treatment [45].…”

A neutrophilic dermatosis following treatment of myelofibrosis with ruxolitinib: An emerging phenomenon?

“…In the last six years, there have been four cases of Sweet syndrome (SS) reported in patients with ruxolitinib‐treated myelofibrosis. (1–4) To our knowledge, this is the first case reported on a ruxolitinib‐associated neutrophilic dermatosis presenting with a pathergy phenomenon at a post‐operative wound site, accompanied by mucosal involvement.…”

A Case Report of Necrotizing Neutrophilic Dermatosis: A Sheep in Wolf’s Clothing

“…The alternate differential diagnosis for an erythematous and/or painful rash in conjunction with systemic toxicity is a necrotizing soft tissue infection (NSTI), a highly lethal condition requiring emergent and often extensive surgical debridement to prevent certain mortality [ 4 ]. Given the rapid progression shared by both NND and NSTI along with their markedly divergent management strategies, parsing out the correct diagnosis is of paramount importance for clinicians [ 3 , 5 , 6 ].…”

“…NND: necrotizing neutrophilic dermatosis; NSTI: necrotizing soft tissue infection[4,5,10,[13][14][15][16].…”