Sweet syndrome: clinical presentation, associations, and response to treatment in 77 patients

J Am Acad Dermatol. 2013 Oct;69(4):557-64. doi: 10.1016/j.jaad.2013.06.023. Epub 2013 Jul 23.

Abstract

Background: Sweet syndrome is a neutrophilic dermatosis with cutaneous tender lesions that can be associated with malignancies, infections, systemic inflammatory disorders, and medications. Although numerous studies have described Sweet syndrome, few studies have systematically investigated Sweet syndrome.

Objective: We sought to describe characteristics and treatments of patients with Sweet syndrome and evaluate clinical differences depending on the underlying cause.

Methods: A retrospective study was conducted to identify patients with Sweet syndrome evaluated at Mayo Clinic from 1992 to 2010.

Results: Of 77 patients with Sweet syndrome (mean age of onset 57 years), 43 (56%) were male. Eighteen patients (23%) reported a preceding infection. A total of 41 (53%) patients were classified as having classic Sweet syndrome, 27 (35%) patients had malignancy-associated Sweet syndrome, and in 9 (12%) patients drug-induced Sweet syndrome was considered. In all, 21 patients had a hematologic malignancy or myeloproliferative/myelodysplastic disorder, whereas 6 patients had solid tumors. The mean hemoglobin level, in both male and female patients (P < .0443 and P < .0035, respectively), was significantly lower in malignancy-associated versus classic and drug-induced Sweet syndrome. Systemic corticosteroids were the most frequently used treatment (70%).

Limitations: This is a retrospective study and represents patients from a single academic center.

Conclusions: Sweet syndrome is a distinctive disorder with certain clinical and histologic characteristics, which usually has a complete response to systemic corticosteroids. It is important to evaluate Sweet syndrome patients who have laboratory evidence of anemia for an underlying malignancy.

Keywords: MDS; MPD; Sweet syndrome; drug-induced Sweet syndrome; malignancy-associated Sweet syndrome; myelodysplastic syndrome; myeloproliferative disorder; treatment.

Publication types

  • Comparative Study

MeSH terms

  • Academic Medical Centers
  • Adjuvants, Immunologic / therapeutic use
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Age Factors
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Anti-Bacterial Agents / therapeutic use
  • Antineoplastic Agents / therapeutic use
  • Cohort Studies
  • Comorbidity
  • Dermatologic Agents / therapeutic use
  • Drug Therapy, Combination
  • Drug-Related Side Effects and Adverse Reactions / epidemiology*
  • Female
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Neoplasms / epidemiology*
  • Neoplasms / pathology
  • Retrospective Studies
  • Risk Assessment
  • Severity of Illness Index
  • Sex Factors
  • Sweet Syndrome / drug therapy*
  • Sweet Syndrome / epidemiology*
  • Sweet Syndrome / etiology
  • Sweet Syndrome / physiopathology
  • Treatment Outcome

Substances

  • Adjuvants, Immunologic
  • Adrenal Cortex Hormones
  • Anti-Bacterial Agents
  • Antineoplastic Agents
  • Dermatologic Agents