Kawasaki disease

One of three oral changes can occur (1) dry, red, swollen and fissured lips, (2) redness of the entire oropharynx, or (3) strawberry tongue. Skin changes of the extremities include swelling of the hands and feet; red palms and soles; generalized red skin with some polymorphic lesions; and peeling of the fingertips from distal to proximal. The fingertip peeling can be recurrent after the febrile phase. Lymph node enlargement greater than 1.5 cm may be noted, especially in cervical lymph nodes. It is particularly seen in toddlers and young children and may cluster in winter and spring outbreaks.

The serious complication of Kawasaki disease is the development of coronary artery aneurysms secondary to segmental vasculitis and are found in 10–20% of affected children. The mortality rate in the USA is 2%. Aneurysms can sometimes be detected by EKG changes but coronary angiography may be required. Kawasaki disease is recurrent in 4% of children. Acute adenovirus infections, leptospirosis, scarlet fever, drug eruptions and toxic shock syndrome may mimic.

Laboratory changes are non-specific with elevated sedimentation rate, C-reactive protein, lactic acid dehydrogenase plus mild anemia and thrombocytosis can be seen.

Intravenous immune globulin (IVIG) and low-dose aspirin if given within 10 days of onset of fever may prevent coronary aneurysms. IVIG is given as a single dose of 2 g/kg or 400 mg/day for 4 days. Children who present with a serum C-reactive protein greater than 10 mg/mL, a lactic acid dehydrogenase greater than 590 IU/mL or a hemoglobin less than 10 g/dL may not respond to IVIG. Retreatment with IVIG may be necessary in patients with persistent fever. High-dose glucocorticosteroids are contraindicated as heart disease may increase in those treated. Early involvement of a pediatric cardiologist is recommended.