A Rare Case of Neonatal Systemic Xanthogranulomatosis With Severe Hepatic Disease and Metachronous Skin InvolvementPapadakis, 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, PhDJournal of Pediatric Hematology/Oncology: April 2012 - Volume 34 - Issue 3 - p 226–228 doi: 10.1097/MPH.0b013e3182203086 Clinical and Laboratory Observations Abstract Author Information 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. Departments of *Pediatric Hematology-Oncology †Pathology ‡Dermatology §Neonatal Intensive Care Unit, “Agia Sofia” Children's Hospital, Athens, Greece The authors declare no conflict of interest.Reprints: Vassilios Papadakis MD, PhD, Department of Pediatric Hematology-Oncology, “Agia Sofia” Children's Hospital, Thivon & Livadias Ave, Goudi, Athens 11527, Greece (e-mail: firstname.lastname@example.org). Received February 26, 2011 Accepted April 18, 2011 Copyright © 2012 Wolters Kluwer Health, Inc. All rights reserved.