Images in Clinical Practice Indian Pediatrics 2005; 42:950 |
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Juvenile Xanthogranuloma |
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JX are benign tumors of histocytic cells, which occur predominately in infancy and early childhood. The etiology is unknown and the tumors represent accumulations of differentiated histocytes of non-Langerhans’ cell type. Histologically an established lesion shows a mixed cellular dermal infiltrate with histocytes. lymphocytes, eosionophils and typical Tuotion cells (giant cells with wreathlike arrangement of nuclei). Fifty percent of cases of JX have been reported in Infants below 6 months. Lesion may occur in children over 3 years of age and cases have been reported in adults. Visceral involvement may occur in lung, liver, spleen, kidney and deeper soft tissues. Eye involvement (10%) may lead to secondary glaucoma. JX has been associated with neurofibromatosis, Neimann-Pick disease, myelogenous leukemia and urticaria pigmentosa. JX can be differentiated from xanthoma by the distribution of the lesion and absence of lipid abnormalities. Two other differential diagnosis include molluscum contagiosum (pearly, dome shaped papule with central umbilication) and neurofibroma (firm lesion with associated Cafe-au-lait spots) . No treatment is necessary for the cutaneous lesion as they are self limiting and lesions resolve by 1-5 years. Only reassurance is needed. Where treatment is indicated as in ocular lesion, surgery or radiotherapy will give good results . T.M. Ananda Kesavan,
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