Also called mucocutaneous lymph node syndrome, this disorder of unknown origin occurs mainly in children under ﬁve and was ﬁrst described in Japan. It is characterised by high fever, conjunctivitis (see under EYE, DISORDERS OF), skin rashes and swelling of the neck glands. After about two weeks the skin from ﬁngertips and toes may peel. The disease may last for several weeks before spontaneously resolving. It is possible that it is caused by an unusual immune response to INFECTION (see IMMUNITY).
Arteritis is a common complication and can result in the development of coronary artery aneurysms (see ANEURYSM) in up to 60 per cent of those aﬀected. These aneurysms and even myocardial infarction (see HEART, DISEASES OF – Coronary thrombosis) are often detected after the second week of illness. The disease can be hard to diagnose as it mimics many childhood viral illnesses, especially in its early stages. The incidence in the UK is over 3 per 100,000 children under ﬁve years of age.
Treatment Because of the danger of coronary artery disease, prompt treatment is important. This is with intravenous IMMUNOGLOBULINS and low-dose aspirin. To be eﬀective, treatment must start in the ﬁrst week or so of the illness – a time when it is most diﬃcult to diagnose.