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Sweet’s syndrome, or acute febrile neutrophilic dermatosis,[1] is a condition characterized by the sudden onset of fever, leukocytosis, and tender, erythematous, well-demarcated papules and plaques which show dense neutrophilic infiltrates on histologic examination.
It is named for Robert Douglas Sweet.[2][3]
Although it may occur in the absence of other known disease, Sweet’s syndrome is often associated with hematologic disease (including leukemia), and immunologic disease (rheumatoid arthritis, inflammatory bowel disease).
A genetic association has been suggested,[4] but no specific genetic link has been identified.
Sweet, working in Plymouth in 1964, described a disease with four features: fever; leukocytosis; acute, tender, red plaques; and a papillary dermal infiltrate of neutrophils. This led to the name acute febrile neutrophilic dermatosis. Larger series of patients showed that fever and neutrophilia are not consistently present. The diagnosis is based on the two constant features, a typical eruption and the characteristic histologic features; thus the eponym Sweet’s syndrome (SS) is used.
Sweet’s syndrome can be classified based upon the clinical setting in which it occurs: classical or idiopathic Sweet’s syndrome, malignancy-associated Sweet’s syndrome, and drug-induced Sweet’s syndrome.[5]
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