The Subtle Storm: Identifying Incomplete Kawasaki Disease in Children

Abstract Incomplete Kawasaki disease (KD) poses a significant diagnostic challenge, particularly in pediatric patients who do not fulfill the classical diagnostic criteria[1]. Timely recognition and early initiation of therapy are crucial to prevent serious cardiovascular complications[2], especially coronary artery involvement.It is the leading cause of acquired heart disease in children in developed countries. The highest incidence is in Asia with almost 1 in a 100 children in Japan having the disease by age 5 [2]. In Africa, two cases were reported in South Africa in Caucasian children in 1980 but the first case in an African child was reported in Ivory Coast in 1981. Despite of sporadic cases being reported across many countries in Africa, mainly North Africa and the West African region, the epidemiological data for KD is limited for African countries [3,4,5]. The etiology of the disease remains unknown and several hypothesis exist in trying to explain the cause. Epidemiologic data suggest that an infectious agent may be causing the disease in genetically susceptible individuals [6]. The classic presentation of the disease occurs in children below 5 years. This suggests that there is an environmental trigger to which children mount an immune response after which the disease no longer manifests [7]. Postulated infectious agents include variants of normal flora which are induced by environmental factors such as improved hygiene [8]. Pathogenetic-mechanisms postulated to result in endothelial injury include that of a protein homeostasis system in which immune cells target pathogenic proteins bound to endothelial cells resulting in host cell injury [9]We present a case of a 6-year-old child with prolonged fever, mucocutaneous manifestations, and elevated inflammatory markers, ultimately diagnosed with incomplete KD. Prompt treatment with intravenous immunoglobulin (IVIG) led to rapid clinical improvement and prevented coronary complications. This case underscores the importance of maintaining high clinical suspicion and initiating early management in children with atypical or incomplete presentations of Kawasaki disease. KEYWORDS: Incomplete Kawasaki Disease, Pediatric vasculitis, IVIG, Coronary artery complications, Maculopapular rash.