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Treatment of juvenile xanthogranuloma
Author(s) -
Stover Daniel G.,
Alapati Srilatha,
Regueira Osvaldo,
Turner Curtis,
Whitlock James A.
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21523
Subject(s) - juvenile xanthogranuloma , medicine , langerhans cell histiocytosis , histiocyte , histiocytosis , pathology , dermatology , chemotherapy , diabetes insipidus , disease , surgery , pediatrics
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. Pediatr Blood Cancer 2008;51:130–133. © 2008 Wiley‐Liss, Inc.