HistoryThis section has been translated automatically.
Mac Intyre, 1850; Kahler, 1889
DefinitionThis section has been translated automatically.
Rare, primary cutaneous (extramedullary) B-cell NHL lymphoma with a low malignancy grade (see below lymphoma, cutaneous B-cell lymphoma) without (primary detectable) systemic involvement.
Primary plasmacytomas of the skin are very rare.
Secondary (metastatic) manifestations of multiple myeloma are somewhat more common. They usually occur in scarring structures (e.g., sternotomy scars; Li A et al. ) and are associated with a much worse prognosis than primary cutaneous plasmacytomas(Yoo J et al.).
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EtiopathogenesisThis section has been translated automatically.
Unknown; extramedullary plasmocytomas are frequently found after organ transplants. Individual reports exist about an EBV association.
ManifestationThis section has been translated automatically.
The average age is 70 years
LocalizationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
HistologyThis section has been translated automatically.
Nodular or diffuse tumour cell aggregates in the entire dermis and possibly in subcutaneous fatty tissue. Mostly atypical plasmacytoid cells, mature or immature plasma cells; more rarely immunoblasts. No epidermotropy. Intracellular: immunoglobulins, often monoclonal IgA synthesis and more rarely IgG synthesis. In PAS preparations, PAS-positive inclusions in the cytoplasm (Russel corpuscles).
DiagnosisThis section has been translated automatically.
Histological and immunohistological clarification of the findings is important, as well as the exclusion of systemic involvement (extremely accelerated BSG, 1-h value:>100mm n.W., so-called fall reduction, proteinuria with L-chain excretion = (Bence-Jones proteins; Bence-Jones proteins are found in about 60% of all multiple myelomas from IgG or IgA light-chain myeloma). Low-dose CT is recommended as the most sensitive imaging method. Skeletal scintigraphy is less suitable because myeloma foci often do not store data. MRI and PET are suitable for extramedullary foci.
Differential diagnosisThis section has been translated automatically.
Note(s)This section has been translated automatically.
The term plasma cytoma is only valid for the solitary monorganic infestation of an organ, in the absence of bone marrow infiltration.
Low-malignant B-cell NHL, as a systemic disease with mostly multilocular or diffuse penetration of the bone marrow by malignant cloned plasma cells, is called"multiple myeloma".
Only in the case of systemic infestation (multiple myeloma) do the following parameters become diagnostically relevant: BSG elevation, paraproteinemia, paraproteinuria (Bence-Jones sample), also cryoglobulins. X-ray skull: mothlike osteolysis (Note: myeloma cells do not have osteolytic activities of their own; however, they stimulate osteolysis by means of various osteoporosis types). Cytokines (RANKL =receptor activator of NF-kB-ligand, MIP-1 = macrophage inflammatory protein = CCL3) stimulate osteoclast and inhibit osteoblast activity. Plasmacellular bone marrow infiltrations, cryoglobulinemia; extremely elevated BSG.
LiteratureThis section has been translated automatically.
- Bayer-Garner IB et al (2003) The spectrum of cutaneous disease in multiple myeloma. J Am Acad Dermatol 48: 497-507
- Cerroni L, Kerl H (2003) Cutaneous B-cell lymphomas. In: Kerl H et al (eds.) Histopathology of the skin. Springer Publishing House, Berlin Heidelberg New York S. 908-910
- Gómez-Armayones S et al (2015 Cutaneous Nodules in Multiple Myeloma. Cutaneous plasmacytoma associated with multiple myeloma. ActasDermosifiliogr
- Kahler O (1889) On the symptomatology of multiple myeloma. Observation of albumosuria. Prague medical weekly (Prague) 14: 33-35 and 44-49
- Kazakov DV (2002) Primary cutaneous plasmacytoma: a clinicopathological study of two cases with a long-term follow-up and review of the literature. J Cutan Pathol 29: 244-248
- Landthaler M, Scherer R, Wolff HH (1980) Cryoglobulinemia in plasmocytoma. dermatologist 31: 661-667
- Li A et al.(2013) Primary cutaneous plasmacytoma occurring after pacemaker implantation andrecurring
in scar tissue. Dermatol Online J 19:3.
- MacIntyre W (1850) Case of mollities and fragilitas ossium. Medico-Surgical Transactions (London) 33: 211-232
- Maejima H et al (2014) Multiple primary cutaneous plasmacytoma. Acta dermatovenerol Croat 22:308-310
- Santos G et al (2014) Case for diagnosis. Cutaneousinvolvement associated to multiple myeloma. An Bras Dermatol 89:173-174
- Yoo J et al (2017) Cutaneous Plasmacytoma: Metastasis of Multiple Myeloma at the Fracture Site.
Ann Dermatol 29:483-486.
- Wilms K (1987) Non-Hodgkin lymphomas. In: Siegenthaler W, Kaufmann W, Hornbostel H, Waller HD (Ed.) Textbook of Internal Medicine. Thieme Stuttgart New York. S. 630–633
- Wuu A et al (2014) Primary cutaneous plasmacytoma. Cutis.93:E19- 21
Incoming links (7)Beta2-microglobulin; Cd classification; Cutaneous plasmocytoma; Immunodeficiency syndrome variable; Lichen myxoedematosus (classic type); Multiple myeloma; Primary cutaneous marginal zone lymphoma;
Outgoing links (10)Ccl3; Cd138; Cd20; Cd38; Cd45; Cd79a; Cutaneous b-cell lymphomas (overview); Paraneoplastic syndromes (overview); Pseudotumour of cutaneous inflammatory; Skeletal scintigraphy;
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.