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Journal of the American Academy of Dermatology
Volume 3, n° 3
pages 257-270 (septembre 1980)
Doi : 10.1016/S0190-9622(80)80189-7
Clinical and laboratory studies

Necrobiotic xanthogranuloma with paraproteinemia

Steven Kossard, M.B., Ph.D. , R.K. Winkelmann, M.D., Ph.D.
 From the Department of Dermatology, Mayo Clinic and Mayo Foundation, USA 

1Reprint requests to: Dr. Steven Kossard, c/o Section of Publications, Mayo Clinic, 200 First St., SW, Rochester, MN 55901.

Eight patients had multiple xanthomatous plaques and subcutaneous nodules that had a predilection for the periorbital area, flexures, and trunk and that tended to ulcerate. Skin biopsy specimens showed a combination of xanthogranulomatous nodules with necrobiosis. All patients had an accompanying dysproteinemia, which was a monoclonal IgG paraprotein in six. Hyperlipidemia, low serum complement, and cryoglobulinemia were variable features. Five patients had leukopenia. Bone marrow examination showed myeloma in two patients, a lymphoproliferative process in one, and some atypical plasma cells in two. Cutaneous necrobiotic xanthogranuloma is a distinctive histologic pattern most frequently related to plasma cell dyscrasias, and it should be distinguished from normolipemic plane xanthoma and other necrobiotic granulomas.

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* The immunoglobulin studies and paraprotein characterization were performed in the laboratory of Robert A. Kyle, M.D., and the immunoperoxidase studies, by Peter M. Banks, M.D.

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