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Necrobiotic xanthogranuloma - Skin
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Necrobiotic xanthogranuloma - Skin

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Contributors: Renata Prado MD, Whitney A. High MD, JD, MEng, Jeffrey D. Bernhard MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Necrobiotic xanthogranuloma (NXG) is a rare histiocytic disorder that presents as firm yellow papules and plaques of the skin, most often in the periorbital area. It is strongly associated with an IgG monoclonal gammopathy (80% to 90% of cases), particularly IgG kappa (60%). NXG may affect extra-cutaneous tissues including the spleen, heart, lung, kidney, intestine, ovary, larynx, pharynx, skeletal muscle, and central nervous system.

In a review of 48 cases of NXG, the mean age of onset was the sixth decade, with ages ranging from 17 to 85 years. The condition demonstrates no sex predilection.

NXG often follows a protracted and indolent course, but patients with the condition also have an increased risk of plasma cell dyscrasias (multiple myeloma and monoclonal gammopathy of undetermined significance, or MGUS) and other lymphoproliferative disorders. When these co-existing conditions exist, the disease may follow a fatal course.

The etiology of NXG is unknown. It is speculated that the paraprotein, present in the majority of cases, binds to lipoprotein receptors of monocytes, inducing formation of a xanthogranuloma, or may bind to serum lipoproteins.


D76.3 – Other histiocytosis syndromes

404164003 – Necrobiotic xanthogranuloma

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Differential Diagnosis & Pitfalls

The clinical differential diagnosis for NXG may include the following:
  • Necrobiosis lipoidica – more common in diabetic patients and nearly always occurs on the pretibial area; this condition does not have an association with paraproteinemia.
  • Granuloma annulare – classically occurs as annular lesions without scale; usually erythematous without the yellow color seen in NXG.
  • Xanthelasma – occurs as thin yellow plaques most often near the inner canthus of the eyelid, more often on the upper lid than the lower lid. Xanthelasma, in contrast to NXG, is usually thin and soft, without extension into the orbit or other ocular symptoms.
  • Normolipemic plane xanthoma – yellow macules or thin plaques that may also be associated with monoclonal gammopathy but usually occur on the on neck, flexural areas, and less often the face.

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Last Updated: 07/10/2013
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Necrobiotic xanthogranuloma - Skin
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Necrobiotic xanthogranuloma : Eyelids, IgG elevated, Plaque with ulcer, Smooth plaque, Yellow color, WBC decreased, RBC decreased
Clinical image of Necrobiotic xanthogranuloma
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