Necrobiotic xanthogranuloma with paraproteinemia

J Am Acad Dermatol. 1980 Sep;3(3):257-70. doi: 10.1016/s0190-9622(80)80189-7.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Antineoplastic Agents / therapeutic use
  • Blood Protein Electrophoresis
  • Female
  • Granuloma / complications*
  • Granuloma / drug therapy
  • Granuloma / pathology
  • Humans
  • Immunoglobulin G
  • Male
  • Middle Aged
  • Paraproteinemias / etiology*
  • Paraproteinemias / pathology
  • Skin / pathology
  • Xanthomatosis / complications*
  • Xanthomatosis / drug therapy
  • Xanthomatosis / pathology


  • Antineoplastic Agents
  • Immunoglobulin G