Acute febrile cholestasis as an inaugural manifestation of Kawasaki's disease

We report a child who developed acute febrile cholestasis with jaundice and pruritus as the inaugural manifestation of Kawasaki's disease (KD). The se vere obstructive icterus and hydrops of the gallbladder required cholecyste ctomy that was not followed by remission of the fever and cholestasis. KD w as suspected after the exclusion of all infectious, metabolic and neoplasti c conditions responsible for acute cholestasis. The administration of intra venous gammaglobulin (IVGG) promptly induced defervescence and improvement of the patient's general condition. Mucocutaneous alterations, peeling of the digits, right cervical lymph node enlargement and bilateral non-suppurative conjunctivitis supporting the di agnosis of KD developed 14 days after the appearance of jaundice. No corona ry abnormalities had developed after 2 years of follow-up. We conclude that this syndrome should be suspected in any child with febril e cholestasis of unknown origin, in order that coronary involvement may be prevented by the administration of IVGG.