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New Painful Nodules in a Patient With Multiple Myeloma

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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C. Plasmacytoma

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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Article Information

Corresponding Author: Edison W. Tsui, MD, Department of Medicine, Dartmouth-Hitchcock Medical Center, One Medical Center Drive, Lebanon, NH 03766 (edison.w.tsui@hitchcock.org).

Published Online: August 10, 2017. doi:10.1001/jamaoncol.2017.2112

Conflict of Interest Disclosure: None reported.

References
1.
Bladé  J, de Larrea  CF, Rosiñol  L.  Extramedullary involvement in multiple myeloma.  Haematologica. 2012;97(11):1618-1619.PubMedGoogle ScholarCrossref
2.
Kois  JM, Sexton  FM, Lookingbill  DP.  Cutaneous manifestations of multiple myeloma.  Arch Dermatol. 1991;127(1):69-74.PubMedGoogle ScholarCrossref
3.
Boyle  EM, Davies  FE, Leleu  X, Morgan  GJ.  Understanding the multiple biological aspects leading to myeloma.  Haematologica. 2014;99(4):605-612.PubMedGoogle ScholarCrossref
4.
Pereira  MA, Baudrier  T, Costa  A, Magalhães  J, Azevedo  F.  Cutaneous metastatic plasmacytomas with tropism for a previously injured limb.  Dermatol Online J. 2008;14(9):16.PubMedGoogle Scholar
5.
Piette  WW.  Myeloma, paraproteinemias, and the skin.  Med Clin North Am. 1986;70(1):155-176.PubMedGoogle ScholarCrossref
6.
Patterson  JW, Parsons  JM, White  RM, Fitzpatrick  JE, Kohout-Dutz  E.  Cutaneous involvement of multiple myeloma and extramedullary plasmacytoma.  J Am Acad Dermatol. 1988;19(5 Pt 1):879-890.PubMedGoogle ScholarCrossref
7.
Araujo  C, Marques  H, Fernandes  JC, Pardal  A, Brito  C.  Cutaneous plasmacytomas secondary to nonsecretory multiple myeloma.  J Dermatolog Clin Res. 2014;2(3):1022.Google Scholar
8.
Fonseca  R, Abouzaid  S, Bonafede  M,  et al.  Trends in overall survival and costs of multiple myeloma, 2000-2014.  [published online January 20, 2017].  Leukemia. 2017. doi:10.1038/leu.2016.380PubMedGoogle Scholar
9.
Nooka  AK, Kastritis  E, Dimopoulos  MA, Lonial  S.  Treatment options for relapsed and refractory multiple myeloma.  Blood. 2015;125(20):3085-3099.PubMedGoogle ScholarCrossref
10.
Park  S, Lee  SJ, Jung  CW,  et al.  DCEP for relapsed or refractory multiple myeloma after therapy with novel agents.  Ann Hematol. 2014;93(1):99-105.PubMedGoogle ScholarCrossref
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