1/5. Intracardiac juvenile xanthogranuloma in a newborn.Juvenile xanthogranuloma (JXG) presents a normolipemic non-langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/5. Juvenile xanthogranuloma: case report with immunohistochemical identification of early and late cytomegalovirus antigens.Juvenile xanthogranuloma (JXG) is a dermatological condition of unknown etiology that rarely affects the oral mucosa. There are conflicting reports suggesting that it may represent a reactive virally-induced lesion associated with cytomegalovirus (CMV) infection. The present paper reports an additional case of oral JXG and discusses its possible association with CMV infection. The biotin-streptavidin system was used to detect early and late CMV antigens. Positive immunolabelling for both antigens was demonstrated in some histiocytes in the lesion. These findings suggest that JXG may be associated with CMV infection.- - - - - - - - - - ranking = 6keywords = antigen (Clic here for more details about this article) |
3/5. Deep juvenile xanthogranuloma: a lesion related to dermal indeterminate cells.Juvenile xanthogranuloma (JXG) is considered to represent a lesion originating from histiocytes. Three cases of deeply located JXG and one case of cutaneous JXG were studied. One case with extensive mesenteric involvement presented with hypercalcemia and one case with liver involvement had hypergammaglobulinemia. immunohistochemistry, electron microscopy, karyotyping, and dna flow cytometry were used to determine the phenotype of the cells involved and to find further clues as to the histogenesis of these lesions. Immunohistochemically, all lesions studied expressed the CD1a antigen but showed no labeling for S-100 protein. The cells did not contain Birbeck granules. From these data it is suggested that the cells involved are of indeterminate dermal histiocyte lineage and that occurrence of deep located lesions of JXG may be due to migration of CD1 a-positive histiocytes.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/5. Juvenile xanthogranuloma with cutaneous and cerebral manifestations in a young infant.Juvenile xanthogranuloma is usually a self-limiting disease of the skin. Intracranial manifestations are extremely rare. We report the clinico-pathological features of an 8-month-old boy suffering from a gradually enlarging nodule of the chest wall and subsequent epileptic seizures. The subcutaneous tumor and a cerebral subcortical tumor of the left temporal lobe were resected. The histological appearance of both tumors corresponded to juvenile xanthogranuloma and included histiocytes, foamy cells, giant cells, inflammatory cells, and collagen-producing fibroblasts showing a storiform pattern. Immunohistochemical studies demonstrated positivity of the tumor cells for lysozyme, CD68 and myeloid-histiocytic antigen, but not S-100 protein, supporting mono-histiocytic differentiation. This case indicates that juvenile xanthogranuloma should be considered in the differential diagnosis of intracranial "xanthomatous" and histiocytic lesions.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/5. Deep juvenile xanthogranuloma: an unusual presentation.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.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |