Necrobiotic xanthogranuloma associated with myeloma

Abstract

We observed a 73-year-old man with necrobiotic xanthogranuloma (NXG) on the eyelids and concurrent multiple myeloma. The treatment was surgery followed by administration of melphalan. No relapse was seen in 19 months of follow-up. We consider that surgical removal of xanthogranuloma is an advisable rapid treatment that involves fewer side-effects than other treatments.

Necrobiotic xanthogranuloma with giant cell hepatitis, successfully treated with intravenous immunoglobulins

“…NXG represents a rare disease of unknown origin characterized by indurated, erythematous to yellowish papules and plaques with preferential involvement of the periorbital area , but affecting also the trunk and extremities . It is frequently associated with paraproteinemia , especially IgG kappa light‐chain monoclonal gammopathy , which, in some cases, progresses to multiple myeloma . Our patient exhibited cutaneous lesions of NXG in a systemic background of paraproteinemia and giant cell hepatitis.…”

A 7-Year History of Necrobiotic Xanthogranuloma following Asymptomatic Multiple Myeloma: A Case Report

“…80–90% of cases demonstrate monoclonal gammopathy (IgG Kappa for 60% and IgG Lambda for 26%); however, only 10% of cases will develop multiple myeloma [2, 12]. Other related conditions that can be found with NXG included Hodgkin's disease, non-Hodgkin's lymphoma, chronic lymphocytic leukemia, myelodysplastic syndrome, macroglobulinemia, cryoglobulinemia, and amyloidosis.…”

“…Cryotherapy and radiotherapy have been tried without satisfied results. Thalidomide may be an interesting option for recalcitrant skin lesions [12, 27]. …”

Necrobiotic Xanthogranuloma

“…Skin lesions can recur rapidly, and lesion size typically increases with recurrence. [5][6][7][8][9] Systemic diseases such as lymphoproliferative diseases and other hematologic disorders also are associated with NXG. 1 Most NXG skin lesions (60%-70%) first appear on the trunk or extremities and subsequently involve the periorbital area.…”

“…Despite these potential complications, incisional biopsy is recommended to confirm the diagnosis when NXG is suspected clinically. 2,7 Approximately 100 cases of NXG have been described in the literature, and most reports emphasize clinical findings. Although 80% to 85% of patients have ocular lesions, 1 these are neither pathognomonic nor required for a diagnosis of NXG.…”