Periorbital necrobiotic xanthogranuloma treated successfully with novel multiple myeloma therapy.

A 46-year-old woman with a medical history of a multinodular goiter presented to the department of dermatology with extensive yellow papules and nodules surrounding both eyelids that had developed over the past 3 years. A skin biopsy was done that showed chronic granulomatous dermal inflammation and foreign body giant reaction consistent with juvenile xanthogranuloma after ruling out a ruptured chalazion. She was subsequently referred to our institute for a comprehensive dermatology evaluation and underwent a repeat biopsy of the skin of the right lateral canthus, which resulted in a diagnosis of necrobiotic xanthogranuloma (NXG, Figure 1). Given its association with underlying plasma cell dyscrasias, a workup was undertaken. The complete blood count (CBC) revealed a white blood count (WBC) of 2.93 × 103/μL, absolute neutrophil count (ANC) of 950 cells/ μL, and hemoglobin level of 16.6 g/dL. The chemistry panel showed plasma protein of 8.6 g/dL, plasma albumin of 4.3 g/dL, creatinine of 0.86 mg/dL, and calcium of 9.2 mg/dL. The serum protein electrophoresis (SPEP) test showed an M-spike in the γ region of 1.64 g/dL, and the serum immunofixation electrophoresis (IFE) test showed IgG κ. Urine protein electrophoresis (UPEP) was negative for Bence-Jones protein. β2 microglobulin was 2.58 mg/dL. Quantitative immunoglobulin testing revealed an elevated IgG of 2180 mg/dL (normal, 751-1560 mg/dL). A bone marrow biopsy revealed plasma cell dyscrasia with hypercellular marrow (60%) and an atypical plasma cell population of 7% of the bone marrow space. Immunohistochemical staining revealed a subset of plasma cells that were positive for BCL-1, CD123, and CD 117-C-KIT. A definite clonal plasma cell population was not detected by immunohistochemical stains for κ and λ light chains on the bone marrow sample, presumably because any small clonal proliferation was obscured by more numerous benign background polytypic plasma cells. However, flow cytometry showed a small population of κ-restricted plasma cells. A skeletal survey was negative for any lytic lesions. Hematologic studies and bone marrow findings were consistent with monoclonal gammopathy of unknown significance (MGUS). Owing to pain associated with the skin lesions and cosmetic concerns, therapy was initiated with lenalidomide (Revlimid, Celgene) 25 mg orally daily for 3 weeks given on a 28-day cycle. After adjusting the lenalidomide dose for neutropenia at the second cycle, dexamethasone 40 mg on days 1, 8, and 15 of a 28-day cycle was added in the third cycle. The patient completed 5 cycles by August Address correspondence to: Omar Al Ustwani, MD, Department of Medicine, Roswell Park Cancer Institute, Elm and Carlton Streets, Buffalo, New York 14263; Phone: 716-903-1244; Fax: 716-845-8008; E-mail: [email protected]. Figure 1. A, Hematoxylin and Eosin [H&E] stain demonstrating dense infiltrate of inflammatory cells in the dermis. B, H&E stain showing characteristic extracellular lipid and cholesterol clefts in acellular necrobiotic debris.