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Chapter 32. Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)

Philip R. Cohen; Herbert Honigsmann; Razelle Kurzrock

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Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome) at a Glance
  • Acute febrile neutrophilic dermatosis (Sweet syndrome) is characterized by a constellation of symptoms and findings: the acute onset of fever, neutrophilia, erythematous, and tender skin lesions that typically show an upper dermal infiltrate of mature neutrophils, and the prompt improvement of both symptoms and lesions after initiation of treatment with systemic corticosteroids.
  • Cardiovascular, central nervous system, gastrointestinal, hepatic, musculoskeletal, ocular, oral, otic, pulmonary, renal, and splenic organs can be involved by the extracutaneous manifestations of Sweet syndrome.
  • Classical Sweet syndrome may be associated with infection of the upper respiratory tract and/or gastrointestinal tract, inflammatory bowel disease, and pregnancy.
  • Malignancy-associated Sweet syndrome occurs in individuals with previously undiagnosed or relapsing hematologic malignancies and solid tumors; in these patients, Sweet syndrome appears as a cutaneous paraneoplastic syndrome.
  • Drug-induced Sweet syndrome describes the onset of dermatosis in patients following the initiation of certain medications—most commonly granulocyte-colony stimulating factor.
  • Cytokines—directly or indirectly—may have an important etiologic role in the pathogenesis of this dermatosis.
  • The first-line oral systemic agents for treating Sweet syndrome are corticosteroids, potassium iodide, and colchicine.
  • The second-line oral systemic agents for treating Sweet syndrome are indomethacin, clofazimine, cyclosporine, and dapsone.
  • Topical application of high-potency corticosteroids or intralesional corticosteroids may be efficacious for treating localized Sweet syndrome lesions.

Acute febrile neutrophilic dermatosis was originally described by Dr. Robert Douglas Sweet in the August–September 1964 issue of the British Journal of Dermatology. The cardinal features of “a distinctive and fairly severe illness” that had been encountered in eight women during the 15-year period from 1949 to 1964 were summarized. Although the condition was originally known as the Gomm–Button disease “in eponymous honor of the first two patients” with the disease in Dr. Sweet's department, “Sweet's syndrome” has become the established eponym for this acute febrile neutrophilic dermatosis.1–10

More than 1,000 cases of Sweet syndrome have been reported since Sweet's original paper.1–509 The distribution of Sweet syndrome cases is worldwide and there is no racial predilection.1,2,12,16–20,30,31 The dermatosis presents in three clinical settings.13,15

Diagnostic criteria for classical or idiopathic Sweet syndrome were proposed by Su and Liu in 1986 and modified by von den Driesch in 1994 (Table 32-1).11–14 It may be associated with infection (upper respiratory tract or gastrointestinal tract), inflammatory bowel disease, or pregnancy.13,15 Two studies have noted a seasonal preference for the onset of Sweet syndrome for either autumn or spring in 70% of 42 patients.416 or autumn.496

Table 32-1 Diagnostic Criteria for Classical Sweet Syndrome versus Drug-Induced Sweet Syndrome
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Table 32-1 Diagnostic Criteria for Classical Sweet Syndrome versus Drug-Induced Sweet Syndrome

Classicala

Drug Inducedb

(1) Abrupt onset of painful erythematous plaques ...

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