Lymphomatoid granulomatosis in a 4-year-old boy

Lymphomatoid granulomatosis is a necrotizing angiocentric and angiodestruct ive infiltrative process involving primarily the lung, skin, central nervou s system, and kidney. The incidence is highest in middle-aged men and is ra re in children. We report a case of lymphomatoid granulomatosis involving b oth skin and lung in a 4-year-old boy. The disease progressed to peripheral T-cell lymphoma, which was unusual in light of recent evidence suggesting a a-cell origin in the majority of cases.