🔍 Search
ParaneoplasticPeriorbitalICD-10 D76.3

Necrobiotic xanthogranuloma

NXG; necrobiotic xanthogranuloma with paraproteinaemia

Necrobiotic xanthogranuloma is a rare granulomatous disorder characterised by yellow-orange, indurated, often ulcerated plaques and nodules — most strikingly in a periorbital distribution — associated in approximately 80% of cases with a monoclonal gammopathy (almost always IgG, more often Îș than λ). The paraproteinaemia may precede, coincide with or follow the skin disease by years, and progression to multiple myeloma occurs in a substantial minority. Diagnosis requires histological demonstration of dermal-subcutaneous palisading necrobiosis with multinucleated giant cells (including Touton-type) and cholesterol clefts, together with documented paraproteinaemia. Treatment is directed at the underlying haematological disorder; chlorambucil, IVIg, lenalidomide and autologous stem-cell transplantation have all been used.

CurrentLast reviewed 15 May 2026

Clinical features

  • Yellow-orange to red-brown, indurated plaques and nodules; often atrophic centre with telangiectasias; ulceration in approximately 40–50%.
  • Periorbital distribution highly characteristic — up to 80% of cases; trunk, flexures and extremities also affected.
  • Median age 55–65; female predominance.
  • Lesions slowly progressive over years; multiple new lesions over time.
  • Ocular complications — episcleritis, scleritis, keratitis, uveitis — in a minority.

Haematological associations

  • Monoclonal gammopathy in ~ 80% — almost always IgG (Îș > λ) by serum protein electrophoresis / immunofixation.
  • Multiple myeloma in 10–25% over follow-up.
  • MGUS or smouldering myeloma at presentation in many; lymphoproliferative disease occasionally.
  • Cryoglobulinaemia, hyperlipidaemia, hypocomplementaemia, hepatosplenomegaly may co-exist.

Histology

  • Dermal-subcutaneous palisading granulomas around broad zones of necrobiotic collagen.
  • Multinucleated giant cells — characteristic Touton-type giant cells and bizarre foreign-body-type giant cells.
  • Cholesterol clefts within necrobiotic zones — a distinguishing feature from necrobiosis lipoidica.
  • Plasma cells, lymphocytes, foamy histiocytes; lymphoid follicles may be present.
  • Differential — necrobiosis lipoidica (often shins of diabetics, no cholesterol clefts), granuloma annulare (smaller, less necrobiotic), xanthoma disseminatum.

Workup

  • Histology — skin biopsy with adequate depth to include subcutis.
  • Serum protein electrophoresis and immunofixation (Îș:λ ratio).
  • FBC, U&E, LFT, calcium, ÎČ2-microglobulin, lipid profile, glucose.
  • Bone-marrow biopsy and skeletal survey for myeloma assessment if paraproteinaemia confirmed.
  • Ophthalmology review.
  • Haematology referral.

Management

  • Treatment of the underlying paraproteinaemia drives skin disease response.
  • First-line — chlorambucil (low-dose alkylating agent); intralesional / systemic corticosteroids.
  • Other reported therapies — IVIg, melphalan-prednisolone, thalidomide, lenalidomide, rituximab (in selected B-cell-driven disease), autologous stem-cell transplantation for myeloma.
  • Topical / intralesional steroids and tacrolimus for symptomatic plaques.
  • Surgical excision of disfiguring lesions usually unsatisfactory — recurrence common.
  • Long-term haematological surveillance — risk of progression to multiple myeloma remains lifelong.

References

  1. Wood AJ et al. Necrobiotic xanthogranuloma — a review of 17 cases. Arch Dermatol; 2009.
  2. Hilal T et al. Necrobiotic xanthogranuloma — treatment review. Hematol Oncol; 2018.

Spot a correction?

If any clinical statement, citation or link on this page needs updating, please email admin@skinoncology.net with the page name, the proposed correction and the supporting source.