Exploring the atypical: a case of atypical Kawasaki disease in a 9-year-old Filipino male with down syndrome

Abstract

Kawasaki disease (KD) is an acute systemic vasculitis of the small- and medium-sized arteries. Due to its predilection for the coronary arteries, there is a potential for the development of coronary artery aneurysms and subsequent risk of sudden death. Down syndrome is a less-frequently occurring comorbidity in KD, as reported in a Japanese nationwide survey in 2017. A 9-year-old Filipino male with Down syndrome was referred to dermatology service due to generalized erythematous plaques and oral mucosal changes, accompanied with intermittent high-grade fever, and not responding to cefuroxime IV at 64mg/kg/day, paracetamol IV at 10mg/kg, and cetirizine 10mg/day. Further workup revealed dilated right coronary artery on two-dimensional echocardiography. Patient was then managed as a case of atypical Kawasaki disease, and was started on aspirin 80mg/tab ½ tab once daily. It is essential to consider atypical Kawasaki disease in patients having features of KD and yet not meeting the criteria for classic KD and to promptly start proper treatment in order to avoid development of coronary artery aneurysms and subsequent risks. Despite being a less-frequently occurring comorbidity in KD, patients with Down syndrome may also present with incomplete or atypical KD with coronary artery abnormalities.