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Abstract:  We report a 9-month-old girl and a 4-year-old boy with acute febrile neutrophilic dermatosis (Sweet syndrome). Both children were febrile, had leukocytosis, and exhibited lesions characteristic of Sweet syndrome. Both had an antecedent infection. Our evaluation and long-term follow-up of these children failed to reveal evidence of underlying malignancy or a chronic systemic illness typically encountered in Sweet syndrome. Of interest, the 4-year-old boy responded to systemic corticosteroids with remission, whereas the 9-month-old infant experienced flaring of the disease on successive attempts to taper the systemic corticosteroids. Systemic corticosteroid usage was associated with alteration in behavior in the 4-year-old and transient growth retardation in the 9-month-old. In both patients, the adverse effects resolved after discontinuation of the corticosteroids.