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Aim: Initial presentation with only cervical lymphadenopathy and fever is one of the pitfalls in the diagnosis of Kawasaki disease (KD). As the number of such patients is small, their clinical features have remained uncertain. The purpose of the present study is to characterise the features of such KD patients, especially in comparison with those of patients with common onset., Methods: We conducted a retrospective review of the medical records of 136 consecutive KD patients admitted to Kobe City General Hospital from April of 2000 to March of 2006. Twenty-nine of the 136 patients initially presented with only cervical lymphadenopathy and fever and were classified into the lymphadenopathy-KD (LKD); they were compared with the remaining 107 KD patients with other presentations (other-KD)., Results: Age, days of fever to diagnosis, and duration of fever were significantly higher or longer in LKD patients, who also showed higher C-reactive protein levels and neutrophil alkaline phosphatase activity. There were no significant differences between two groups in gender, duration of hospitalization, frequency of high-dose intravenous immunoglobulin (IVIG) administration, coronary artery lesions (CALs), white blood cell or platelet counts, and levels of hemoglobin or albumin on admission., Conclusions: Although a delay in diagnosis and stronger inflammation were found in LKD patients, such differences did not have any significant effect on patients' outcomes as assessed by the frequency of IVIG administration and the presence of CALs.