Sweet syndrome (SS), the prototypical neutrophilic dermatoses (ND), is characterized by abrupt onset of tender plaques and nodules, classically accompanied by fever and leukocytosis. While management mainly relies on systemic corticosteroids, inadequate response can be seen in some patients that necessitates exploring other treatment options. Early diagnosis of malignancy-associated SS along with detection of concomitant malignancy is crucial for improving patients’ outcomes. Data regarding various clinical manifestations, extracutaneous associations, treatment and outcomes are poorly characterized in literature. We aimed to review all published case reports and case series to portray clinical features of SS including extracutaneous manifestations. We also describe reported treatment options and their outcomes to draw attention towards unmet therapeutic needs in the management of SS. In addition, for clinical and practical purposes, we attempted to delineate the distinction between malignancy-associated SS (MA-SS) and non-malignant subtypes of SS.
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