Acute febrile neutrophilic dermatosis (Sweet syndrome) is rare in children and is regularly associated with underlying malignancies or inflammatory diseases. A 5-year-old girl with glycogen storage disease type Ib, neutropenia, and recurrent infections developed characteristic skin eruption of Sweet syndrome after 2 years of granulocyte colony-stimulating factor (G-CSF) therapy. This association points to a possible role of G-CSF-induced granulopoiesis and granulocyte activation in the pathogenesis of Sweet syndrome.

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