title: Deep juvenile xanthogranuloma: an unusual presentation.
abstract: Juvenile xanthogranuloma (JXG) is a disorder of histiocytes usually associated with cutaneous lesions. It may present a diagnostic dilemma in the absence of cutaneous lesions and when deeply located. Differentiation of JXG from other childhood histiocytosis syndromes, especially Langerhans' cell histiocytosis (LCH), is important. We describe an unusual case of deep JXG in a 27-month-old girl with multiple omental and peritoneal nodules presenting with ascites. Although a diagnosis of LCH was suspected clinically, the absence of Birbeck granules and S-100 protein and T6 antigen negativity, together with CD68 and factor XIIIa positivity, led us to a diagnosis of JXG. Physicians should be aware of the widening spectrum of manifestations of juvenile xanthogranuloma.
authors.affiliation: Department of Pathology, Long Island Jewish Medical Center, New Hyde Park, NY 11040, USA.
authors.forename: U
authors.initials: U
authors.lastname: Neveling
authors.forename: L B
authors.initials: LB
authors.lastname: Kahn
authors.forename: E
authors.initials: E
authors.lastname: Valderrama
authors.forename: M
authors.initials: M
authors.lastname: Poustchi-Amin
authors.forename: D
authors.initials: D
authors.lastname: Uckan
authors.forename: A
authors.initials: A
authors.lastname: Shende
mesh_terms: D000005:Abdomen; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D000945:Antigens, Differentiation, T-Lymphocyte / Q000378:metabolism; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008854:Microscopy, Electron; D009418:S100 Proteins / Q000378:metabolism; D014057:Tomography, X-Ray Computed; D011503:Transglutaminases / Q000378:metabolism; D014972:Xanthogranuloma, Juvenile / Q000175:diagnosis* / Q000378:metabolism / Q000473:pathology*; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D000945:Antigens, Differentiation, T-Lymphocyte; D009418:S100 Proteins; D011503:Transglutaminases; C067980:CD68 antigen, human; D000097382:CD68 Molecule