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A woman in her 60s presented for evaluation of multiple painful cutaneous nodules that had developed over the previous 2 months. The patient had a history of IgGκ multiple myeloma, diagnosed approximately 6 months earlier. At that time, flow cytometry showed a κ-restricted plasma cell population positive for CD38 and CD138 but negative for CD3, CD20, and CD45 expression. Cytogenetic analysis with fluorescent in situ hybridization did not reveal high-risk genetic markers. The patient demonstrated no evidence of end organ disease, and her myeloma was classified as stage II. Since her initial diagnosis, she had completed 4 cycles of lenalidomide, bortezomib, and dexamethasone (RVD) therapy in addition to irradiation for lytic lesions of the thoracic and lumbar spine. Physical examination revealed scattered red-violaceous nodules, with the largest measuring approximately 6.0 × 5.5 × 2.5 cm (Figure, A). They were found in the mouth, the trunk, and on all 4 extremities. The lesions were nonulcerated, round with a smooth surface, fixed, and firm and tender to touch. Serum protein levels were normal. An ultrasound-guided needle biopsy and immunohistochemical analysis of the largest lesion on the patient’s right shoulder were performed (Figure, B and C).
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Positron emission tomography–computed tomography demonstrated innumerable foci of hypermetabolic activity within the subcutaneous soft tissue but without evidence of direct medullary extension. Biopsy showed diffuse involvement of a monotypic κ-restricted plasma cell dyscrasia with necrosis (Figure, B). The tissue core sample showed extensive necrosis; however, nonnecrotic regions demonstrated a dense monomorphic infiltrate of primarily immature atypical cells with a plasmacytoid appearance. Immunohistochemical stains revealed diffuse CD138 positivity (Figure, C) but negativity for CD3, CD20, CD56, BCL6, and c-kit. These findings were consistent with the patient’s history of κ-restricted multiple myeloma.
The development of her cutaneous and mucosal plasmacytomas, despite 4 cycles of RVD, was concerning for refractory disease, and the treatment regimen was switched to dexamethasone, cyclophosphamide, etoposide, and cisplatin (DCEP). The patient received the first cycle of DCEP (day 1-4) as an inpatient without complications. No skin breakdown or ulceration was noted during chemotherapy. At her 1-week follow-up visit, the cutaneous plasmacytomas were found to be smaller, less firm in consistency, and reportedly less tender.
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Corresponding Author: Edison W. Tsui, MD, Department of Medicine, Dartmouth-Hitchcock Medical Center, One Medical Center Drive, Lebanon, NH 03766 (firstname.lastname@example.org).
Published Online: August 10, 2017. doi:10.1001/jamaoncol.2017.2112
Conflict of Interest Disclosure: None reported.
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