title: Treatment of juvenile xanthogranuloma.
abstract: Juvenile xanthogranuloma (JXG) is generally a benign, self-limited histiocytic disorder of the skin. We report two cases of multisystem JXG presenting with clinical features more commonly seen in Langerhans cell histiocytosis (LCH), including diabetes insipidus and lytic bony lesions. Histologically, the skin lesions demonstrated a histiocytic dermal infiltrate that stained for CD-68, but S-100 and CD1a stains were negative. Treatment according to LCH-based chemotherapy regimens resulted in prompt resolution of symptoms. A literature review of multisystem JXG cases treated with chemotherapy suggests that symptomatic patients can successfully be treated with LCH-based regimens that include both corticosteroids and vinca alkaloids.
authors.affiliation: Vanderbilt University School of Medicine, Nashville, Tennessee, USA.
authors.forename: Daniel G
authors.initials: DG
authors.lastname: Stover
authors.forename: Srilatha
authors.initials: S
authors.lastname: Alapati
authors.forename: Osvaldo
authors.initials: O
authors.lastname: Regueira
authors.forename: Curtis
authors.initials: C
authors.lastname: Turner
authors.forename: James A
authors.initials: JA
authors.lastname: Whitlock
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis; D006801:Humans; D007223:Infant; D008297:Male; D016896:Treatment Outcome; D014972:Xanthogranuloma, Juvenile / Q000175:diagnosis / Q000188:drug therapy*