Necrobiotic xanthogranuloma: a systemic disease with paraproteinemia

A woman of 39 presenting with a conjunctival swelling with a necrobiotic xanthogranulomatous histology suffered from a series of destructive cutaneous lesions of similar histology for 20 yr. She had a paraproteinemia and developed myelomatosis. At postmortem xanthogranulomatous lesions were found in skin, larynx, heart and kidneys. The patient also had nodular transformation of the liver with portal hypertension.