Necrobiotic xanthogranuloma with paraproteinemia without periorbital involvement - A case report

Necrobiotic xanthogranuloma is an uncommon granulomatous disease involving the skin and extracutaneous tissues. It is characterized by indurated, yellow-red plaques and nodules, involving primarily the face and less frequently the trunk and extremities. The disease has a strong association with paraproteinemia and other hematologic or lymphoproliferative disorders. Histologically, the dermal part shows xanthogranulomatous change with extensive necrobiosis and many Touton and foreign-body giant cells. Here, we describe a case of a 46-year-old man with a 1-year history of multiple cutaneous lesions over the trunk and thighs. Necrobiotic xanthogranuloma was diagnosed by histology and clinically associated with paraproteinemia. This case is also unusual in that there was no periorbital involvement, which is believed to be a typical feature of this disease.