title: Erdheim-Chester disease in childhood: a challenging diagnosis and treatment.
abstract: Erdheim-Chester disease is a rare, non-Langerhans systemic histiocytosis characterized by bilateral sclerosis of the metaphyseal regions of the long bones and infiltration in other organs. The histopathologic hallmark is defined by a mononuclear infiltrate of foamy histiocytes and rare pathognomonic Touton giant cells with extensive fibrosis. This condition is exceptional in children. We report here a case of Erdheim-Chester disease in a 10-year-old girl with retroperitoneal infiltration and bone involvement, for whom the diagnosis was only established after a 3-year course with multiple biopsies. It is also the first pediatric case successfully treated with interferon-alpha suggesting that interferon-alpha can be a safe and efficient first-line therapy for this disease in children.
authors.affiliation: Department of Pediatrics, Pediatric Rheumatology, Bicêtre University Hospital, Le Kremlin Bicêtre, France. tu-anh.tran@bct.aphp.fr
authors.forename: Tu-Anh
authors.initials: TA
authors.lastname: Tran
authors.forename: Monique
authors.initials: M
authors.lastname: Fabre
authors.forename: Daniele
authors.initials: D
authors.lastname: Pariente
authors.forename: Irina
authors.initials: I
authors.lastname: Craiu
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.forename: Frederic
authors.initials: F
authors.lastname: Charlotte
authors.forename: Pierre
authors.initials: P
authors.lastname: Eid
authors.forename: Antoine
authors.initials: A
authors.lastname: Durrbach
authors.forename: Yassine
authors.initials: Y
authors.lastname: Taoufik
authors.forename: Isabelle
authors.initials: I
authors.lastname: Kone-Paut
mesh_terms: D001847:Bone Diseases; D002465:Cell Movement; D002648:Child; D031249:Erdheim-Chester Disease / Q000175:diagnosis* / Q000188:drug therapy*; D005260:Female; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use; D012074:Remission Induction
chemical_list: D016898:Interferon-alpha