ICD-11 classes
14 Diseases of the skin
Skin disorders involving specific cutaneous structures
Disorders of cutaneous blood and lymphatic vessels
EF40 — Vasculitis or capillaritis involving the skin
4A44.5 — Mucocutaneous lymph node syndrome

ICD-11 4A44.5 — Mucocutaneous lymph node syndrome

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.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Vasculitis #3669
• Vasculitis #18397