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 .
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