4A44.5

ICD-11 MMS 2026-01

Mucocutaneous lymph node syndrome

Description

Mucocutaneous lymph node syndrome (Kawasaki disease) is a globally distributed acute vasculitis of young children affecting medium to small calibre arteries and if untreated leads to coronary artery aneurysms in about a quarter of cases. Cardinal signs include cervical lymphadenopathy, conjunctival injection, rash (maculopapular, erythrodermic or erythema multiforme-like), strawberry tongue, oropharyngeal erythema, erythema and swelling of hands and feet and periungual desquamation. The cause is unknown but thought to be environmental, possibly from viral infection. A variant of the disease has been linked to SARS CoV-2 infection.

Index terms

  • Mucocutaneous lymph node syndrome
  • Acute febrile mucocutaneous lymph node syndrome
  • Kawasaki disease
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  • Kawasaki syndrome

Inclusions

  • Kawasaki syndrome

Exclusions from above levels

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A44.0 – Rhizomelic pseudopolyarthritis
  • 4A44.1 – Aortic arch syndrome
  • 4A44.2 – Giant cell arteritis
  • 4A44.3 – Single organ vasculitis
  • 4A44.4 – Polyarteritis nodosa
  • 4A44.6 – Sneddon syndrome
  • 4A44.7 – Primary angiitis of the central nervous system
  • 4A44.8 – Thromboangiitis obliterans
  • 4A44.9 – Immune complex small vessel vasculitis
  • 4A44.A – Antineutrophil cytoplasmic antibody-associated vasculitis
  • 4A44.B – Leukocytoclastic vasculitis
  • 4A44.Y – Other specified vasculitis
  • 4A44.Z – Vasculitis, unspecified

Foundation entity

Mucocutaneous lymph node syndrome – http://id.who.int/icd/entity/540285662

Part of: 4A44: Vasculitis › 04: Diseases of the immune system