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A case of Letterer-Siwe disease in a 6 month-old Korean male manifesting maculopapular skin rash, watery diarrhea, fever and bony skull defect as classical features of the disease was presented. The diagnosis was confirmed by clinical symptoms, histopathologic and radiologic findings. The authors report a fatal case which was treated in hospital with intensive antibiotics, corticosteroid and alkylating agent.
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