Sweet syndrome in patients with and without malignancy: A retrospective analysis of 83 patients from a tertiary academic referral center

Abstract

Background: Sweet syndrome is a neutrophilic dermatosis that may be categorized into classic, malignancy-associated, and drug-induced subtypes. Few studies have systematically analyzed this rare disorder.

Objective: To describe the clinicopathologic characteristics and treatment of Sweet syndrome and identify characteristics associated with concurrent malignancy.

Methods: We retrospectively reviewed patients with Sweet syndrome at the University of Pennsylvania from 2005 to 2015.

Results: We identified 83 patients (mean age, 57 years; 51% male) with Sweet syndrome: 30% with the classic form, 44% with the malignancy-associated form, 24% with the drug-induced form in the setting of malignancy, and 2% with the drug-induced form. Acute myeloid leukemia was the most common malignancy (in 24 of 83 patients [29%]). Filgrastim was the most common medication (used in 8 of 83 patients [10%]). Leukopenia (P < .001), anemia (P = .002), thrombocytopenia (P < .001), absence of arthralgia (P < .001), and histiocytoid or subcutaneous histopathology (P = .024) were associated with malignancy (χ² test).

Limitations: This was a retrospective study that represents patients from a single tertiary academic referral center, which may limit its generalizability to other settings.

Conclusion: When caring for patients with Sweet syndrome, dermatologists should be aware of the potential association of leukopenia, anemia, thrombocytopenia, absence of arthralgia, and histiocytoid or subcutaneous histopathology with malignancy.

Keywords: Sweet syndrome; acute febrile neutrophilic dermatosis; acute myeloid leukemia; fms-like tyrosine kinase 3; karyotype; neutrophil; neutrophilic dermatosis; somatic mutations.