Necrobiotic xanthogranuloma with monoclonal gammopathy of the IgG lambda type.

Abstract

A 77-year-old Swiss woman presented with a 25-year history of smoldering disseminated normolipemic plane xanthomas confined to the neck and elbows accompanied by a monoclonal gammopathy of the IgGλ type. There was a sudden onset of a progressive course with increase in number and size of inflammatory lesions over the past year. The clinical and histological picture supported the development into a necrobiotic xanthogranuloma (NXG). Skin lesions responded to therapy with low-dose melphalan. Our case supports the notion that normolipemic plane xanthoma and NXG form a spectrum of xanthomatous dermal reactions with paraproteinemia.