Sweet-Syndrom

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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 .

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