Introduction Kawaski disease (KD) is the second most common vasculitis of the childhood. The clinical criteria for KD are fever, conjunctivitis, mucositis, rash, extremity changes, and cervical lymphadenopathy. The patient who do not fulfil the classic criteria are diagnosed with incomplete KD after excluding some conditions that associate unexplained fever. Echocardiography can be useful in supporting the diagnosis.

Methods and Results The authors present the case of a little girl, 1 year of age that presented to our clinic for daily unexplained fever that was lasting for 8 days. The general practitioner recommended treatment with cefaclor but the child developed a generalised rash that disappeared after i.v steroid administration. Because the fever persisted she was admitted in a regional hospital for investigations. The laboratory tests revealed marked inflammation, leukocytosis with normal chest x-ray and normal abdominal ultrasound, she received treatment with Ceftriaxon. During the admission she continued having fever and she developed a bilateral conjunctivitis that lasted for 3 days. She was sent to our clinic for additional tests. On admission she had fever but a normal clinical examination. The laboratory tests revealed marked inflammation, leukocytosis, thrombocytosis, normocytic, normochromic anaemia, with sterile blood, urine and naso-pharyngeal cultures, normal CMV, EBV, toxoplasma, HIV, hepatitis antibodies, normal ANA and rheumatoid factor. During admission she developed a rash that lasted for 1 day. We performed an echocardiography that revealed no aneurysm but a small pericardic effusion and signs of coronary arteritis (perivascular brightness). We decided to start treatment with i.v immunoglobulins and aspirin. The fever disappeared after 1 day and an epithelial desquamation was noted on the feet. After 7 days the inflammation disappeared, she had normal leukocytes and slightly elevated thrombocytes. She was discharged with normal clinical examination.

Conclusions AHA/AAP recommend for the diagnosis of incomplete KD and initiation of treatment the following criteria: elevated C-reactive protein and/or erythrocyte sedimentation rate and three or more abnormal supplemental laboratory findings (leukocytosis, normocytic, normochromic anaemia, thrombocytosis, elevated ALT, hypoalbuminemia) or abnormal echocardiography. Children with incomplete KD are at risk of cardiovascular sequelae mostly due to delay of diagnosis and treatment.