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Sweet syndrome

Sweet syndrome (acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include:

  • fever
  • neutrophilia
  • tender erythematous skin lesions (papules, nodules, and plaques)
  • a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis

Pathology

The syndrome presents in three clinical settings:

  • classical (idiopathic) Sweet syndrome (CSS)
    • usually presents in women between the age of 30-50 years and is often preceded by an upper respiratory tract infection
    • it may be associated with inflammatory bowel disease and pregnancy
    • approximately one-third of patients with CSS experience recurrence of dermatosis
  • malignancy-associated Sweet syndrome (MASS)
    • can occur as a paraneoplastic syndrome in patients with an established cancer or individuals whose Sweet syndrome-related hematologic dyscrasia or solid tumor was previously undiscovered
    • MASS is most commonly related to acute myelogenous leukemia
  • drug-induced Sweet syndrome (DISS)
    • most commonly occurs in patients who have been treated with granulocyte-colony stimulating factor, however, other medications may also be associated with DISS

History and etymology

It is named after Dr Robert Douglas Sweet who first described the syndrome in 1964 2.

Article information

rID: 15919
Section: Syndromes
Synonyms or Alternate Spellings:
  • Sweet's syndrome
  • Acute febrile neutrophilic dermatosis

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