Abstract

Xanthoma disseminatum is a rare non-Langerhans' histiocytosis of older children and adults with characteristic lesions involving the skin, mucous membrane and occasionally internal organs. We describe a case, presenting in infancy, with unique clinical features. The patient presented at 8 months of age with skin lesions subsequently found to have histologic, immunophenotypic, and ultrastructural characteristics of non-Langerhans' histiocytosis. In addition to extensive skin lesions, the patient also has involvement of the buccal mucosa, lips, eyelids, bones, bone marrow, and possibly liver and spleen. Clinical and pathological features of the patient are suggestive of xanthoma disseminatum. Treatment with cytotoxic, immunomodulatory, and lipid-lowering agents has been unsuccessful to date. Unique characteristics of this case of xanthoma disseminatum include the patient's young age, lytic bone lesions, and previously undescribed bone marrow involvement.

Full Text
Paper version not known

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call

Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.