Review
Sweet's syndrome and malignancy

https://doi.org/10.1016/0002-9343(87)90229-4Get rights and content

Abstract

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is characterized by pyrexia, neutrophilia, and the abrupt appearance of erythematous, painful, cutaneous plaques, primarily on the upper extremities, head, and neck. Histologically, the salient feature is a dense dermal infiltrate of neutrophils. Approximately 10 to 15 percent of published cases of Sweet's syndrome occurred in patients with cancer. This report reviews the 39 patients with malignancy-associated Sweet's syndrome described in the world literature and compares Sweet's syndrome in cancer patients with the idiopathic form of the disease. The most common associated malignancy was acute myelogenous leukemia. However, other myeloproliferative disorders, lymphoproliferative disorders, myelodysplastic syndrome, and carcinomas have been observed. Importantly, the diagnosis of Sweet's syndrome was often the presenting sign of a new or recurrent tumor. The presence of anemia, abnormal platelet counts, immature cells in the differential, and/or severe vesiculobullous or ulcerative cutaneous lesions is infrequent in idiopathic Sweet's syndrome and should alert physicians to the possibility of a more serious underlying disease. Extracutaneous manifestations may occur and most often involve the musculoskeletal system. Response to systemic steroids is dramatic in virtually all patients, regardless of the presence of malignancy.

References (63)

  • PG Hazen et al.

    Acute febrile neutrophilic dermatosis in children: report of two cases in male infants

    Arch Dermatol

    (1983)
  • S Itami et al.

    Sweet's syndrome in infancy

    Br J Dermatol

    (1980)
  • JC Klock et al.

    Febrile neutrophilic dermatosis in acute myelogenous leukemia

    Cancer

    (1976)
  • N Saxe et al.

    Acute febrile neutrophilic dermatosis (Sweet's syndrome): four case reports

    S Afr Med J

    (1978)
  • DA Gunawardena et al.

    The clinical spectrum of Sweet's syndrome (acute febrile neutrophilic dermatosis)—a report of eighteen cases

    Br J Dermatol

    (1975)
  • RE Krauser et al.

    The arthritis of Sweet's syndrome

    Arthritis Rheum

    (1975)
  • NE Driban et al.

    Oral manifestations of Sweet's syndrome

    Dermatologica

    (1984)
  • Y Maekawa et al.

    A case of acute febrile neutrophilic dermatosis

    J Dermatol

    (1984)
  • JI Spector et al.

    Sweet's syndrome

    JAMA

    (1980)
  • M Matta et al.

    Sweet's syndrome: systemic associations

    Cutis

    (1973)
  • PH Cooper et al.

    Acute febrile neutrophilic dermatosis (Sweet's syndrome) and myeloproliferative disorders

    Cancer

    (1983)
  • KD Crow et al.

    Acute febrile neutrophilic dermatosis: Sweet's syndrome

    Dermatologica

    (1969)
  • N Imamura et al.

    Early onset of daunomycin cardiotoxicity in a case of acute myelogenous leukemia associated with Sweet's syndrome

    Hiroshima J Med Sci

    (1985)
  • MR Leibowitz et al.

    Unusual aspects of febrile neutrophilic dermatosis (Sweet's syndrome)

    Afr Med J

    (1982)
  • C Pirard et al.

    Syndrome de Sweet et leucemie myeloide aigue

    Ann Dermatol Venereol

    (1977)
  • SS Raimer et al.

    Febrile neutrophilic dermatosis in acute myelogenous leukemia

    Arch Dermatol

    (1978)
  • RM Soderstrom

    Sweet's syndrome and acute myelogenous leukemia: a case report and review of the literature

    Cutis

    (1981)
  • DK Goette

    Acute myelogenous leukemia presenting as Sweet's syndrome

    J Assoc Milit Dermatol

    (1984)
  • G Tikjob et al.

    Acute febrile neutrophilic dermatosis and abnormal bone marrow chromosomes as a marker for pre-leukemia

    Acta Derm Venereol

    (1985)
  • A Nagler et al.

    Sweet's syndrome: a presenting sign of acute myelogenous leukemia

    Isr J Med Sci

    (1985)
  • TJ Harrist et al.

    Neutrophilic eccrine hidradenitis: a distinctive type of neutrophilic dermatosis associated with myelogenous leukemia and chemotherapy

    Arch Dermatol

    (1982)
  • Cited by (177)

    • Clonal neutrophil infiltrates in concurrent Sweet's syndrome and acute myeloid leukemia: A case report and literature review

      2018, Cancer Genetics
      Citation Excerpt :

      According to its etiology or clinical setting, SS can be classified as classical (or idiopathic), drug-associated and malignancy-associated (MA-SS). MA-SS accounts for 15–20% of all SS cases, and is mainly associated with hematological neoplasms such as myeloid dysplasia syndrome (MDS) and acute myeloid leukemia (AML) [2]. MA-SS related to hematological disorders may present prior to or concurrently with the diagnosis of hematological neoplasms, namely paraneoplastic SS, or as drug-associated SS due to the medication used during treatment like granulocyte-colony stimulating factor (G-CSF) [3].

    • Papules, Nodules, and Ulcers

      2018, Principles and Practice of Pediatric Infectious Diseases
    View all citing articles on Scopus

    Current address: Wistar Institute, 3601 Spruce Street, Philadelphia, Pennsylvania 19104.

    View full text