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AT-A-GLANCE
Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by a constellation of symptoms and findings: the acute onset of fever, neutrophilia, tender erythematous skin lesions that typically show mature neutrophils in the upper dermis, and—after initiation of systemic corticosteroids—a prompt improvement of both symptoms and lesions.
Extracutaneous manifestations of Sweet syndrome can include cardiovascular, central nervous system, gastrointestinal, hepatic, musculoskeletal, ocular, oral, otic, pulmonary, renal, and splenic organs.
Infection of the upper respiratory tract and/or gastrointestinal tract, inflammatory bowel disease, and pregnancy may be associated with classical Sweet syndrome.
In individuals with previously undiagnosed or relapsing hematologic malignancies and solid tumors, malignancy-associated Sweet syndrome may occur as a cutaneous paraneoplastic syndrome.
The onset of the dermatosis in patients following the initiation of certain medications—drug-induced Sweet syndrome—is most commonly associated with granulocyte colony-stimulating factor.
The pathogenesis of Sweet syndrome remains to be established; cytokines—directly or indirectly—may have an important etiologic role.
Corticosteroids, potassium iodide, and colchicine are the first-line oral systemic agents for treating Sweet syndrome.
Indomethacin, clofazimine, cyclosporine, and dapsone are the second-line oral systemic agents for treating Sweet syndrome.
Localized Sweet syndrome lesions may be effectively treated with the topical application of high-potency corticosteroids or intralesional corticosteroids.
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Sweet syndrome (also known as acute febrile neutrophilic dermatosis) is typically characterized by the acute onset of pyrexia and painful cutaneous lesions that are composed of a dense dermal inflammatory infiltrate of mature neutrophils. Neutrophilia is also frequently present. Both the condition-associated symptoms and the dermatosis-related lesions promptly resolve after initiation of treatment with systemic corticosteroids.1
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HISTORICAL PERSPECTIVE
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Dr. Robert Douglas Sweet, originally described acute febrile neutrophilic dermatosis in the August-September 1964 issue of the British Journal of Dermatology. He summarized the cardinal features of “a distinctive and fairly severe illness” that had been encountered in 8 women during the 15-year period from 1949 to 1964. In Dr. Sweet’s department, the condition was originally referred to as the Gomm-Button disease “in eponymous honor of the first two patients” with the disease. Subsequently, this acute febrile neutrophilic dermatosis has become best known by the eponym “Sweet syndrome.”1-3
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Numerous individuals with Sweet syndrome have been reported. There is no racial predilection and the distribution of Sweet syndrome cases is worldwide.1 The dermatosis may present in various clinical settings: classical, malignancy-associated, and drug-induced (Tables 36-1 and 36-2).4-7
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