Juvenile Xanthogranuloma With Extracutaneous Lesions

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Abstract
A case of juvenile xanthogranuloma is reported with involvement of the skin and pericardium. The patient had the skin lesions from birth, but presented at 3 months of age with bleeding from the perocardial site producing hemopericardium. The skin and pericardial lesions were biopsied and radiation therapy was given to the pericardium. The child is otherwise in good health. Three other cases of systemic involvement with juvenile xanthogranuloma have been reported in the literature, along with an increasing number of cases with involvement of the eye. Despite these patients with extracutaneous lesions, juvenile xanthogranuloma mainly involves the skin, and should be considered as a benign reactive process which undergoes frequent xanthomatization. It should not be considered a part of the systemic reticuloendotheliosis.

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