XANTHOMA DISSEMINATUM IN AN INFANT WITH SKELETAL AND MARROW INVOLVEMENT

Purpose: Xanthoma disseminatum is a rare non-Langerhans' histiocytosis of older children and adults with characteristic lesions involving th e skin, mucous membrane and occasionally internal organs. We describe a case, presenting in infancy, with unique clinical features. Patient and Methods: The patient presented at 8 months of age with skin lesion s subsequently found to have histologic, immunophenotypic, and ultrast ructural characteristics of non-langerhans' histiocytosis. In addition to extensive skin lesions, the patient also has involvement of the bu ccal mucosa, lips, eyelids, bones, bone marrow, and possibly liver and spleen. Results: Clinical and pathological features of the patient ar e suggestive of xanthoma disseminatum. Treatment with cytotoxic, immun omodulatory, and lipid-lowering agents has been unsuccessful to date. Conclusion: Unique characteristics of this case of xanthoma disseminat um include the patient's young age, lytic bone lesions, and previously undescribed bone marrow involvement.