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Sweet syndrome

Disease definition

A rare inflammatory disease characterized by abrupt appearance of painful, edematous and erythematous papules, plaques and nodules on the skin, and frequently accompanied by fever and neutrophilia with a dense infiltration of mature neutrophils that are typically located in the upper dermis. The disease is classically associated with inflammatory disease, pregnancy, infection (mostly of the upper respiratory tract), or vaccination but may be idiopathic, associated with a hematological or visceral malignancy, or drug-induced.


Classification level: Disorder
  • Synonym(s):
    • Acute febrile neutrophilic dermatosis
  • Prevalence: Unknown
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: Adult
  • ICD-10: L98.2
  • ICD-11: EB20
  • OMIM: 608068
  • UMLS: C0085077
  • MeSH: D016463
  • GARD: 521
  • MedDRA: 10000748

Detailed information

Disease review articles

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.