ICD-11

Vasculitides represent a heterogenous group of diseases of multifactorial aetiology characterised by inflammatory lesions of vessels. These lesions consist of fibrinoid necrosis (necrotizing arteritis), giant cell infiltration without necrosis, immunoglobulins deposit or leukocytoclastic infiltration. The spectrum and severity of the systemic vaculitides is broad, from life or sight threatening fulminant disease to relatively minor skin disease.

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.