Necrobiotic xanthogranuloma with type 1 cryoglobulinemia mimicking necrobiosis lipoidica in a young woman with myeloma

Necrobiotic xanthogranuloma (NXG) is a rare condition currently classified as a non–Langerhans cell histiocytosis and associated with an underlying paraproteinemia (most commonly IgG-κ) in approximately 80% of patients.1 Recent diagnostic criteria have been proposed, including 2 major criteria of clinical and histopathologic features consistent with NXG and 2 minor criteria of paraproteinemia, plasma cell dyscrasia, and/or other associated lymphoproliferative disorder and periorbital distribution of cutaneous lesions in the absence of a foreign body, infection, or other identifiable cause.

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