Normolipemic plane xanthoma (NPX) is a well-characterized clinicopatho
logic entity distinct from necrobiotic xanthogranuloma (NXG). We prese
nt the case of a patient with the classic clinical course of NPX but w
ith many histologic features in common with NXG, namely, multinucleate
d lipophages, cholesterol clefts, and degeneration of collagen with as
sociated cellular necrosis (necrobiosis). We suggest that NPX and NXG
represent part of a spectrum of xanthomatous dermal reactions associat
ed with paraproteinemias and may be more closely related than previous
ly recognized.