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Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e3182203086
Clinical and Laboratory Observations

A Rare Case of Neonatal Systemic Xanthogranulomatosis With Severe Hepatic Disease and Metachronous Skin Involvement

Papadakis, Vassilios MD, PhD*; Volonaki, Eleni MD, PhD*; Katsibardi, Katerina MD, PhD*; Stefanaki, Kalliopi MD, PhD; Valari, Manthoula MD, PhD; Anagnostakou, Marina MD, PhD§; Polychronopoulou, Sophia MD, PhD*

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Abstract

Juvenile xanthogranuloma (JXG) is a rare benign disorder of unknown pathogenesis, usually a self-limited condition. Extracutaneous systematic involvement is infrequent. We report one of the few documented cases of congenital systemic JXG, presenting with fever, jaundice, hepatosplenomegaly, ascites, pancytopenia, and delayed skin involvement. Liver biopsy established the diagnosis and JXG was not demonstrated in the bone marrow. Rapid deterioration of liver disease and pancytopenia, prompted us to administer immunosuppressive treatment (Langerhans cell histiocytosis-II Protocol). The patient's clinical condition improved and visceral and skin lesions showed gradual involution. The patient is still free of disease 4 years after the initial presentation.

© 2012 Lippincott Williams & Wilkins, Inc.

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