I report the case of a 68-year-old man who presented with necrobiotic xanthogranuloma (NXG) with concomitant scleroderma, along with the presence of cryoglobulins and cryofibrinogen in the blood. Autoamputation of the distal fingers and parts of the ears also occurred, over time, with exposure to cold temperatures. This is an interesting constellation of findings in this rare disorder that raises questions concerning its pathogenesis. A review of the literature is included.
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