Sweet's syndrome (acute febrile neutrophilic dermatosis),

https://doi.org/10.1016/S0190-9622(94)70215-2Get rights and content

Acute febrile neutrophilic dermatosis, first described in 1964 by Robert Douglas Sweet, has been termed Sweet's syndrome. Classic Sweet's syndrome occurs in middle-aged women after a nonspecific infection of the respiratory or gastrointestinal tract. Raised erythematous plaques with pseudoblistering and occasionally pustules occur on the face, neck, chest, and extremities, accompanied by fever and general malaise. Involvement of the eyes, joints, and oral mucosa as well as internal manifestations of Sweet's syndrome in the lung, liver, kidneys, and central nervous system has been described. The disease is thought to be a hypersensitivity reaction. Parainflammatory (e.g., infections, autoimmune disorders, vaccination) and paraneoplastic (e.g., hemoproliferative disorders, solid malignant tumors) occurrence is found in approximately 25% of the cases and 2% are associated with pregnancy. Sweet's syndrome responds rapidly to systemic therapy with corticosteroids but recurs in about 25% of the cases. Alternative treatment modalities (e.g., potassium iodide, colchicine, dapsone, clofazimine, cyclosporine) have also been used. This article presents data from 38 patients with Sweet's syndrome and reviews its epidemiology, clinical spectrum, histologic features, laboratory results, differential diagnosis, pathogenic mechanisms, associated diseases, and treatment.

Learning objective: At the conclusion of this learning activity participants should be able to outline the essential features of Sweet's syndrome, including aspects of epidemiology, histology, diagnosis, and management of the condition.

References (362)

  • RH Duerr et al.

    Anti-neutrophil cytoplasmic antibodies in ulcerative colitis

    Gastroenterology

    (1991)
  • S Fourman

    Inflammatory glaucoma associated with Sweet's syndrome

    Am J Ophthalmol

    (1988)
  • RD Sweet

    An acute febrile neutrophilic dermatosis

    Br J Dermatol

    (1964)
  • CH Whittle et al.

    Recurrent neutrophilic dermatosis of the face: a variant of Sweet’s syndrome

    Br J Dermatol

    (1968)
  • KD Crow et al.

    Acute febrile neutrophilic dermatosis: Sweet's syndrome

    Dermatologica

    (1969)
  • M Harms

    Sweet-Syndrom (akute febrile neutrophile Dermatose)

    Zentralbl Haut Geschl

    (1981)
  • L Shapiro et al.

    Sweet's syndrome (acute febrile neutrophilic dermatosis)

    Arch Dermatol

    (1971)
  • MJ Matta et al.

    Sweet's syndrome: systemic associations

    Cutis

    (1973)
  • JC Klock et al.

    Febrile neutrophilic dermatosis in acute myelogenous leukemia

    Cancer

    (1976)
  • WPD Su et al.

    Diagnostic criteria for Sweet's syndrome

    Cutis

    (1986)
  • J Wenning

    Akute febrile neutrophile Dermatose: Sweet-Syndrom

    Hautarzt

    (1978)
  • PH Cooper et al.

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

    Cancer

    (1983)
  • RM Soderstrom

    Acute febrile neutrophilic dermatosis (Sweet's syndrome) and internal malignancy

    Semin Dermatol

    (1984)
  • RE Scully et al.

    Case records of the Massachusetts General Hospital. Case 30-1990

    N Engl J Med

    (1990)
  • JS Storer et al.

    Sweet's syndrome

    Int J Dermatol

    (1983)
  • E Zamora Martinez et al.

    Sweet's syndrome: a study of 10 cases and review of the literature

    Rev Clin Esp

    (1990)
  • P Collins et al.

    Acute febrile neutrophilic dermatosis in childhood (Sweet's syndrome)

    Br J Dermatol

    (1991)
  • JP Callen

    Cutaneous vasculitis and other neutrophilic dermatoses

    Curr Opin Rheumatol

    (1993)
  • PR Cohen et al.

    Malignancy-associated Sweet's syndrome: review of the world literature

    J Clin Oncol

    (1988)
  • PR Cohen et al.

    Sweet's syndrome in patients with solid tumors

    Cancer

    (1993)
  • DA Gunawardena et al.

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

    Br J Dermatol

    (1975)
  • L Hommel et al.

    The incidence of Sweet's syndrome in Geneva: a retrospective study of 29 cases

    Dermatology

    (1993)
  • J Smolle et al.

    Acute febrile neutrophilic dermatosis (Sweet syndrome): a retrospective clinical and histological analysis

    Hautarzt

    (1990)
  • J Delabie et al.

    Histiocytes in Sweet's syndrome

    Br J Dermatol

    (1991)
  • C Butzmann

    Akut febrile neutrophile Dermatose(Sweet-Syndrom)

    (1992)
  • D Sitjas et al.

    Acute febrile neutrophilic dermatosis (Sweet's syndrome)

    Int J Dermatol

    (1993)
  • N Saxe et al.

    Acute febrile neutrophilic dermatosis (Sweet's syndrome)

    S Afr Med J

    (1978)
  • S Itami et al.

    Sweet's syndrome in infancy

    Br J Dermatol

    (1980)
  • M Vittori et al.

    Syndrome de Sweet chez l’enfant

    Lyon Med

    (1980)
  • RV Attili et al.

    Sweet’s syndrome in a child

    Med J Zambia

    (1983)
  • PG Hazen et al.

    Acute febrile neutrophilic dermatosis in children

    Arch Dermatol

    (1983)
  • A Munoz Hoyos et al.

    Sweet’s syndrome in an infant

    Pédiatrie

    (1983)
  • AG Kibbi et al.

    Acute febrile neutrophilic dermatosis (Sweet's syndrome): case report and review of the literature

    Pediatr Dermatol

    (1985)
  • AG Kibbi et al.

    Acute febrile neutrophilic dermatosis (Sweet's syndrome): case report and review of theliterature

    Pediatr Dermatol

    (1985)
  • TC Edwards et al.

    Sweet's syndrome with multifocal sterile osteomyelitis

    Am J Dis Child

    (1986)
  • J Keller et al.

    Sweet-Syndrom mit Immunkomplex-vaskulitis bei einem Kind

    Z Hautkr

    (1986)
  • R Litt et al.

    Sweet syndrome in early childhood

    Eur J Pediatr

    (1986)
  • JH Arndt

    Sweet's syndrome and chronic recurrent multifocal osteomyelitis [Letter]

    Am J Dis Child

    (1987)
  • LR Krilov et al.

    Acute febrile neutrophilic dermatosis (Sweet) presenting as facial cellulitis in a child with juvenile chronic myelogenous leukemia

    Pediatr Infect Dis

    (1987)
  • J Maleville et al.

    Syndrome de Sweet chez un enfant de dix ans

    Ann Dermatol Venereol

    (1987)
  • Cited by (0)

    The CME articles are made possible through an educational grant from the Dermatological Division, Ortho Pharmaceutical Corporation.

    Supported by the Johannes and Frieda Marohn Foundation, DRI-90.

    View full text