International Journal of Clinical & Medical Images

 Clinical presentation A 69 year old woman, with a 1 year history of IgG kappa multiple myeloma (MM), presents with nontender, yellowish lesions and plaques located exclusively on the periorbital region, that have been increasing in size over the past three to four years and were initially misdiagnosed as xanthelasma. Necrobiotic xanthogranuloma (NX) was later suspected and confirmed by hystopathology. The patient was treated with bortezomib plus cyclophosphamide and dexamethasone with very good partial response. The cutaneous lesions were only slightly improved. NX is a chronic granulomatous and xantomathous disease of unknown pathogenesis. The lesions tend to be progressive and the response to chemotherapy is variable and often recur after surgical removal [1]. Hematological disorders are present in up to 80% of cases (IgG monoclonal gammopathy of unknown significance or smoldering myeloma mainly). NX frequently precedes the diagnosis of MM so these patients should be given life-long follow-up [2].