Recurrent Malignancy-Associated Atypical Neutrophilic Dermatosis With Noninfectious Shock

Kathryn Nicole Kinser, Kamaldeep Panach, Arturo Ricardo Dominguez

Research output: Contribution to journalReview articlepeer-review

8 Scopus citations


Sweet syndrome (SS) or acute febrile neutrophilic dermatosis presents with the sudden onset of fever, leukocytosis and tender, erythematous, edematous, well-demarcated papules and plaques that histopathologically demonstrate a dense neutrophilic infiltrate. A total of 20% of patients with SS have malignancy-associated disease that can present with bullous or atypical skin lesions that mimic pyoderma gangrenosum, another neutrophilic dermatosis. Both entities exist on a spectrum, and in the context of underlying malignancy, these neutrophilic diseases become less clinically distinct. The literature also describes life-threatening cases of neutrophilic dermatoses that mimic severe sepsis. We present a fatal case of a patient with chronic eosinophilic leukemia with recurrent episodes of malignancy-associated atypical neutrophilic dermatosis characterized by necrotic skin lesions, pulmonary infiltrates and noninfectious shock and we also summarize the clinical presentations of an additional 10 patients reported in the literature. We conducted a PubMed search of articles published up to and in 2015, focusing on the English and Spanish literature with SS cross-referenced with the following search terms: neutrophilic dermatosis, pyoderma gangrenosum, shock, multiorgan failure and systemic inflammatory response syndrome. The articles were reviewed and the patients׳ clinical and laboratory findings were summarized. Cases of atypical neutrophilic dermatosis presenting with noninfectious shock syndrome are likely underrecognized clinically and underreported in the literature. Patients with malignancy-associated atypical neutrophilic dermatoses associated with noninfectious shock syndrome typically have multisystem disease characterized by recurrent episodes and typically have poor prognoses.

Original languageEnglish (US)
Pages (from-to)626-632
Number of pages7
JournalAmerican Journal of the Medical Sciences
Issue number6
StatePublished - Dec 2017


  • Neutrophilic dermatosis
  • Pyoderma gangrenosum
  • Shock
  • Sweet syndrome
  • Systemic inflammatory response syndrome

ASJC Scopus subject areas

  • Medicine(all)


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