Ann Dermatol.  2020 Feb;32(1):53-56. 10.5021/ad.2020.32.1.53.

Necrobiotic Xanthogranuloma Coexists with Diffuse Normolipidemic Plane Xanthoma and Multiple Myeloma

  • 1Department of Dermatology, Yeungnam University College of Medicine, Daegu, Korea.
  • 2Department of Pathology, Yeungnam University College of Medicine, Daegu, Korea.


Necrobiotic xanthogranuloma (NXG), is a rare multisystem disease that manifests as cutaneous inflammatory lesions, and is commonly associated with lymphoproliferative disease. Diffuse normolipemic plane xanthoma (NX), is also a rare, acquired disease that is often associated with systemic diseases such as lymphoproliferative disease. Both of these diseases have been reported to be associated with monoclonal gammopathy (MG). However, there are few cases in which these diseases co-exist. A 78-year-old female, who had a known case of NX on the neck and axillary area, presented with an asymptomatic erythematous plaque on her left supraclavicular area. Histopathological examination showed lymphoid aggregates, necrobiotic areas, and granulomatous inflammation in the dermis. Numerous foreignbody and Touton type giant cells were noticed. Serum protein immunoelectrophoresis showed an IgG kappa type MG. Lipid profile of the patient was normal. Bone marrow examination showed plasma cell myeloma. Based on these histologic and laboratory results, we diagnosed this lesion as NXG coexisting with NX and multiple myeloma. She was started on treatment with bortezomib and melphalan for multiple myeloma, and high-dose systemic corticosteroid and triamcinolone intralesional injection for the skin lesion. After 3 months of treatment, the NXG skin lesion and MG improved.


Multiple myeloma; Necrobiotic xanthogranuloma; Paraproteinemias; Xanthomatosis

MeSH Terms

Bone Marrow Examination
Giant Cells
Immunoglobulin G
Injections, Intralesional
Multiple Myeloma*
Necrobiotic Xanthogranuloma*
Immunoglobulin G


  • Fig. 1 (A) Well-demarcated yellowish patches and plaques covers the neck, upper chest, and axillary area. (B) Scanning view of biopsy specimen from the yellowish patch lesion. No necrobiosis was seen (H&E, ×40). (C) Many foamy histiocytes (red arrows) were seen in the upper dermis (H&E, ×400).

  • Fig. 2 Dermatologic examination shows kidney-shaped erythematous plaque in the background of yellowish patch on the left supraclavicular area.

  • Fig. 3 (A) Scanning view shows diffuse dermal palisading granuloma (H&E, ×40). (B) Multiple giant cells, either Touton type or Langhan's type, abundant foamy cells, many lymphocytes, and a few plasma cells were seen in the dermis (H&E, ×200). (C) Note that necrobiosis lesions were seen in the dermis (H&E, ×100).

  • Fig. 4 After 3 months treatment of bortezomib, melphalan, high-dose prednisolone, and intralesional triamcinolone injection for Necrobiotic xanthogranuloma and multiple myeloma. The erythematous skin lesion improved in color and thickness, but yellowish skin lesion in the background remained unresponsive to the treatment.


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