EB20

ICD-11 MMS 2026-01

Acute febrile neutrophilic dermatosis

Description

Sweet syndrome (the eponym for acute febrile neutrophilic dermatosis) is characterised by a constellation of clinical symptoms, physical features, and pathological findings which include fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that are typically located in the upper dermis. Sweet syndrome presents in three clinical settings: classical (or idiopathic), malignancy-associated, and drug-induced.

Index terms

  • Acute febrile neutrophilic dermatosis
  • Sweet disease
  • Sweet syndrome
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  • Acute febrile neutrophilic dermatosis, classical type
  • Sweet syndrome, classical type
  • Acute febrile neutrophilic dermatosis, paraneoplastic
  • Sweet syndrome associated with malignancy
  • Sweet syndrome, paraneoplastic
  • Acute febrile neutrophilic dermatosis, drug-induced
  • Sweet syndrome due to drug
  • Neutrophilic dermatosis of the hands
  • Acral Sweet syndrome
  • Neutrophilic dermatosis of the dorsal hands
  • Sweet disease of the dorsa of the hands
  • Atypical pyoderma gangrenosum of the hands

Inclusions

  • Sweet syndrome

Exclusions from above levels

  • 1B72.0 – Bullous impetigo of the napkin area
  • 1F23.12 – Napkin candidosis
  • EH71 – Dermatoses precipitated by drug therapy
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  • EH76 – Dermatoses associated with specific classes of medication

Sibling codes

  • EB21 – Pyoderma gangrenosum
  • EB2Y – Other specified neutrophilic dermatoses

Foundation entity

Acute febrile neutrophilic dermatosis – http://id.who.int/icd/entity/195212152

Part of: 14: Diseases of the skin