title: Extranodal Rosai-Dorfman disease in childhood.
abstract: We describe two children with Rosai-Dorfman disease who presented with exophthalmos, leukocytosis, an elevated sedimentation rate and hypergammaglobulinemia. Both became blind as a result of this condition. One child had associated bilateral cervical lymphadenopathy. Investigation revealed involvement of the nasal fossae and retro-orbital spaces by tumoural masses histologically consistent with the diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML). Immunohistochemical studies suggest that these histiocytes are activated macrophages derived from a phenotype hybrid between "professional" phagocytic cells and immune accessory cells, expressing lysosomal antigens, S-100 protein but rarely, CDla. An underlying immune dysfunction may be central to the pathogenesis of this disease.
authors.affiliation: Institut de Pathologie Clinique, CMU, Geneve, Switzerland.
authors.forename: S
authors.initials: S
authors.lastname: Remadi
authors.forename: I D
authors.initials: ID
authors.lastname: Anagnostopoulou
authors.forename: R
authors.initials: R
authors.lastname: Jlidi
authors.forename: J N
authors.initials: JN
authors.lastname: Cox
authors.forename: T A
authors.initials: TA
authors.lastname: Seemayer
mesh_terms: D018949:Antigens, CD1 / Q000032:analysis; D002648:Child; D015618:Histiocytosis, Sinus / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008198:Lymph Nodes / Q000473:pathology*; D008297:Male; D009296:Nasal Cavity / Q000276:immunology / Q000473:pathology*; D009915:Orbit / Q000276:immunology / Q000473:pathology*; D009418:S100 Proteins / Q000032:analysis
chemical_list: D018949:Antigens, CD1; D009418:S100 Proteins