CLINICS 2012;67(6):689-692 Figure 2 - Symptoms during treatment. Maculopapular skin rash with pruritus distributed over the buttocks and lower extremities was apparent 7 days after fever onset (A, B). Strawberry tongue and non-purulent acute conjunctivitis were apparent 8 days after fever onset (C, D). Periungual desquamation of the fingers was apparent 14 days after fever onset (E, F). exudative tonsillitis, and lymphadenopathy. Although fever and lymphadenopathy are well-known symptoms of KD, the patient’s age and the lack of other initial symptoms consistent with KD led us to consider other diagnoses, such as group A beta-hemolytic streptococcal infection or EBV infection, which also cause prolonged fever, lymphadenopathy, and maculopapular skin rash after treatment with antibiotics. To our knowledge, only one other similar case has been described in the literature, which also involved an older child (8 years) who presented with fever, lymphadenopathy, and tonsillitis (10). There have been several other reports describing patients who presented with tonsillar or peritonsillar symptoms in the absence of lymphadenopathy (11-13). Clinicians need to be aware of the existence of KD and atypical KD and that early treatment is most likely more effective than late treatment. Patients with atypical KD may develop coronary artery damage prior to treatment. Indeed, the atypical presentation of our patient resulted in the delayed diagnosis and initiation of appropriate treatment. Our patient did not meet the classical criteria for the diagnosis of KD until eight days after the onset of fever. Coronary artery aneurysms are relatively common in children with KD who are untreated (14). Our case clearly highlights the importance of early treatment in cases of KD and the need for ongoing echocardiographic monitoring to detect cardiac abnormalities, particularly if the initiation of appropriate treatment has been delayed. Follow-up labora- tory testing should also be performed. 691 Atypical Kawasaki disease Yap C-Y et al. Atypical KD is most common in young infants, who are unfortunately at the greatest risk of developing coronary disease. Identifying such cases can be quite challenging, and fatal outcomes have been reported (5). KD should be considered in the differential diagnosis of prolonged fever in infants. Many physicians who are experienced in the diagnosis and treatment of KD at large medical centers have encountered patients in whom prolonged fever was virtually the sole manifestation of KD (15). In summary, a diagnosis of KD should be considered in children presenting with fever, tonsillitis, and cervical lymphadenopathy, particularly in older children and if empiric antibiotics have proven ineffective. Patients with symptoms suggestive of atypical KD should be referred to a pediatric cardiologist who is experienced in diagnosing this condition. 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