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Kawasaki disease (KD) is an acute febrile vasculitis mainly affecting children, with two types of presentation, namely, typical and atypical. Ït is the most critical cause of coronary artery complications and if not treated on time and appropriately, complications may occur in up to 25% of the patients will get. The occurrence of coronary artery complications increase in atypical presentation of the disease. However, punctual and appropriate treatment will decrease the complications up to 2-4 percent. This study reports four rare cases of incomplete KD. The first one is an 18 month baby girl with prolonged fever and atypical signs and symptoms of KD such as swelling of lateral mid thorax (5 × 10 cm). The second one was a 12-month baby boy presented with a 7-day history of fever without any other symptoms who showed coronary artery involvement in echocardiography. Finally, two cases, one was 4-and the other was 12-years old, who were hospitalized with appendicitis and later were diagnosed as affected by Kawasaki with coronary artery involvement. Âtypical presentation of the disease led to misdiagnosis and prolonged process of diagnosis. Due to the increasing number of atypical Kawasaki cases and the probability of coronary artery disorder, it is recommended that atypical Kawasaki be considered in the differential diagnosis of the disease in the patients with fever of over five days without any clinical cause.

Keywords: Kawasaki disease, atypical Kawasaki, appendicitis, children

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