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Multiple myeloma
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Multiple myeloma

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Contributors: Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Multiple myeloma (MM) is one of two main distinct clonal neoplastic disorders associated with neoplasms of lymphoplasmacytic cells, the other being Waldenström macroglobulinemia. Both disorders have skin manifestations in additional to systemic manifestations, and both begin as a small population of clonal immunoglobulin-producing cells, which then may progress to frank symptomatic malignancies. (An increase in monoclonal proteins without symptoms is referred to as monoclonal gammopathy of undetermined significance [MGUS].) This is a discussion of MM.

In 2010, there were 20 180 new cases of MM in the United States. MM is a disorder of adults (median age of onset 65-70 years) characterized by serum and/or urinary monoclonal proteins, hypercalcemia, anemia (about 80%), lytic bone lesions that are often painful (about 70%), precipitated by movement and often with fractures, decreased renal function (about 25%), and sometimes symptoms related to compression of the spinal cord. Infections, especially with encapsulated pyogenic organisms such as Haemophilus, Staphylococcus, and Streptococcus, are increased in patients with MM. Plasma cells are increased (10% or greater) in the bone marrow. There are some familial tendencies. The disorder is twice as common in Africans and African Americans and 49% more common in males. Incidence increases with age. The disorder most often involves IgG proteins (about one-half of cases), followed by IgA paraproteins (about one-quarter of cases). Some of the proteins aggregate in the cold (cryoglobulins).

POEMS syndrome is characterized by polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes, and sclerotic bone changes resulting from associated MM. Glomeruloid hemangiomas (usually multiple) are relatively specific lesions. Other skin changes include hyperpigmentation, hypertrichosis, skin thickening, and clubbing. Frequent lymphadenopathy with the changes of Castleman disease may occur. Plasma cell leukemia is a rare complication.

Skin lesions of MM may include:
  • Vasculitis and cryoglobulinemia with purpura
  • Amyloidosis
  • Acrocyanosis and livedo reticularis
  • Lipid deposition often precedes myeloma and may be seen in MGUS. IgA myelomas may be more common in xanthomas than in the myeloma population at large. Type III hyperlipidemia is common (with increased cholesterol and triglycerides).
  • Necrobiotic xanthogranuloma
  • Plasma cell infiltration of the skin and plasmacytomas of the skin; sometimes related to the location of needle sticks and bone marrow aspirations.
  • Small, hyperkeratotic-like nasal and facial papules

Codes

ICD10CM:
C90.00 – Multiple myeloma not having achieved remission

SNOMEDCT:
109989006 – Multiple myeloma

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Differential Diagnosis & Pitfalls

The skin manifestations have different differential diagnoses.

Vasculitis and cryoglobulinemia – Lesions often on extremities with purpura and often necrosis; may be angular with geographic or linear borders, suggesting factitial disease. Consider other forms of vasculitis, including drug reactions and hepatitis B and C.

Amyloidosis – Has purpura often without obvious skin lesions.

Xanthomatosis (eg, xanthoma disseminatum, xanthoma tuberosum, xanthoma tendinosum, eruptive xanthoma) – Normolipemic xanthomatosis and hyperlipemic xanthomatosis. Consider other disorders of lipid metabolism.

Necrobiotic xanthogranuloma:
Plasma cell infiltration of the skin and plasmacytoma of the skin

Rarely, or weak association:

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Last Updated: 01/17/2018
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Multiple myeloma
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Multiple myeloma : Hepatomegaly, Bone pain, Creatinine elevated, Lytic bone lesions, Neuropathy peripheral, Numbness, Palpable purpura, Pathologic fracture, Ecchymosis, RBC decreased, Ca elevated
Clinical image of Multiple myeloma
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