Acute febrile mucocutaneous syndrome is an acute systemic vacuities involving medium sized vessels, affecting mainly children under 5 years of age (1). Affect mainly to children 6 months to 4 years of age. This is common in Japan. Young infant more tend to be more severally affected than older children.Cause is unknown, but the many clinical and immunologicalsimilarities with the staphylococcal and streptococcal toxic shock syndromes have led to the suggestion that it is also caused by bacterial toxin acting as a super antigen(2).
Clinical features and diagnosis criteria (2)
Diagnosis is made by mainly clinically. To diagnose (diagnosis criteria) Kawasaki’s disease there should be high grade fever more than 5 days and at least 4 clinical features of following,
Conjunctival injection
Read mucous membranes (Strawberry tongs)
Cervical lymphadenopathy
Rashes
Read and edematous palms, soles
Peeling of fingers and toes
There can be other associated clinical futures like, acute cervical lymphadenopathy, edema of the young, other systemic or local infections(1).
Complications(2)
Affect coronary arteries in 1/3 of children within 1st 6 weeks and then leads to aneurism formation. So, Myocardial ischemia and sudden death can occur. Mortality rate is 1% - 2%. coronary artery aneurysm and sudden death.
Investigations
Full blood count (FBC) and differential count (DC) – thrombocytosis, Leucocytosis
Erythrocyte sedimentary rate (ESR) or C-reactive protein (CRP) - high
Echo cardiogram – aneurismal dilatation of coronary arteries.
Septic screening to exclude other cause of fever
Treatments
Treatment should be started as much as early to minimized complication. If can suspect that child having Kawasaki’s disease all clinical features should not consider to treat patient. Treatment is high dose of Intravenous immunoglobulin and Aspirin. If intravenous immunoglobulin start within first 10 days, risk of coronary artery aneurism formation become low. Aspirin is used to reduce risk of thrombosis. Initially aspirin use as high anti-inflammatory dose until fever is settle and then continue low dose to make antiplatlet action. If patient is not response to immunoglobulin, steroid therapy should start.
Reference –
1. Parveen Kumar, Michael Clark. Clinical medicine, 6th edition: Autoimmune disease(Connective tissue disorder). Christopher P Denton, p583-584
2. Tom Lissauer, Graham Clayden. Illustrated textbook of pediatrics, 3rd edition: Uncommon manifestation of common infection. Dr Saul Faust, Senior lecturer in pediatric, University of Southampton, p237
