[Juvenile xanthogranuloma. Description of a case with liver involvement].
Juvenile Xanthogranuloma. Report of a case with hepatic involvement. The Authors present a case of Juvenile Xanthogranuloma (JX) in a 3 months female child with cutaneous and hepatic nodules associated to dyspnea attributable to obstructive bronchopneumopathy. Histologically the lesions are xanthomatous with proliferation of fat-laden histiocytes. The hepatic involvement is characterized by hepatomegaly and yellow nodules on liver surface as seen at laparoscopy. On liver biopsy there is remarkable expansion of portal triad caused by aggregates of large foamy mono-polynuclear histiocytes with Touton giant cells. The cutaneous nodule biopsy shows histiocytic infiltrate in inter-adnexal dermal space with many giant cells holding great lipidic vacuoles. The patient's follow-up is characterized by slow and progressive clinical improvement with resolution of cutaneous, hepatic and pulmonary pathology. The Authors emphasize the differential diagnosis between this systemic form of JX and Langerhans cell Histiocytosis (Histiocytosis X) with multiorgan involvement. This diagnosis is necessary in order to establish therapy and prognosis.
Di Blasi
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Servizio di anatomia ed istologia-patologica, Ospedale A. Cardarelli di Napoli (USL 40 Regione Campania).
de Seta
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Marsilia
G M
GM
Coletta
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S
Siani
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P
de Rosa
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I
D003937:Diagnosis, Differential; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000175:diagnosis / Q000473:pathology*; D008171:Lung Diseases / Q000175:diagnosis / Q000473:pathology; D012871:Skin Diseases / Q000175:diagnosis / Q000473:pathology*; D014972:Xanthogranuloma, Juvenile / Q000175:diagnosis / Q000473:pathology*
Xantogranuloma giovanile. Descrizione di un caso con interessamento epatico.