Sweet syndrome (acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include:
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
rarely, a steroid-responsive neutrophilic alveolitis which can be fatal 4
Pathology
The syndrome presents in three clinical settings:
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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
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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
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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.