Kawasaki disease shock syndrome is a rare and severe condition related to Kawasaki disease (KD), frequently difficult to diagnose, defined as the presence of any of the following conditions: systolic hypotension (< -2 SD blood pressure defined for age and sex or a decrease in systolic blood pressure from baseline of > 20%) or clinical signs of poor perfusion with accompanying features of KD. We report the case of a 7-year-old girl presenting with atypical KD, complicated by severe shock syndrome and successfully treated with steroids and intravenous immunoglobulins (IVIg).
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