|Year : 2015 | Volume
| Issue : 4 | Page : 172-174
Secondary cutaneous plasmacytoma: A bad prognostic marker
Anuj Bhatnagar1, Rajesh Verma1, JK Bhatia2, Aradhana Sood1, Pankaj Das1
1 Department of Dermatology, AFMC, Pune, Maharashtra, India
2 Department of Pathology, AFMC, Pune, Maharashtra, India
|Date of Web Publication||16-Dec-2015|
Department of Dermatology, AFMC, Pune, Maharashtra
Source of Support: None, Conflict of Interest: None
Plasmacytomas are malignancies of plasma cells that may affect skin or other organs. Plasmacytoma of skin is a rare presentation, which may occur either secondary to multiple myeloma or very rarely may originate in the skin. Histopathology with immunohistochemistry is diagnostic. We present a rare case of secondary cutaneous plasmacytoma.
Keywords: Cutaneous plasmacytoma, plasmacytoma, multiple myeloma
|How to cite this article:|
Bhatnagar A, Verma R, Bhatia J K, Sood A, Das P. Secondary cutaneous plasmacytoma: A bad prognostic marker. J Appl Hematol 2015;6:172-4
| Introduction|| |
Cutaneous Plasmacytomas are monoclonal neoplasms of plasma cells, usually associated with multiple myeloma, resulting either from direct spread from underlying connective tissue focus or from the hematogenous metastatic spread. Cutaneous lesions of multiple myeloma comprises of plasmacytomas, xanthomas, amyloid deposits, vasculitis, calcinosis cutis, and various infections. Primary cutaneous plasmacytomas originate in the skin, without evidence of any underlying primary focus in bone or soft tissue. Appearance of secondary cutaneous plasmacytomas, in particular, is a marker of bad prognosis. We present a case of multiple myeloma with secondary cutaneous plasmacytoma, with a rapid course after onset of skin lesions, resulting in a fatal outcome.
| Case Report|| |
A 60-year-old female, resident of Maharashtra, a known case of multiple myeloma since two and a half years, presented with red, raised skin lesions on body of one and a half month duration. Initially, few pin-head sized lesions appeared in left groin, which gradually increased in size over the next few days to roughly 1 cm, and increased in number and progressed to involve the body, mainly trunk [Figure 1] and [Figure 2]. There was no history of fever, pain, breathlessness, cough, seizures, chest or abdominal pain. Patient had received three cycles of chemotherapy with bortezumab, bendamustin, thalidomide, lenalidomide and melphalan, and a session of radiotherapy for soft tissue mass in the spine, however, there were two episodes of relapse. On presentation, the patient was conscious and oriented. She was afebrile with a pulse of 104/min, blood pressure of 96/68 mmHg and respiratory rate of 24/min. General examination revealed pallor, and enlargement of cervical and axillary lymph nodes, which were firm, mobile and nontender. Systemic examination revealed hepatosplenomegaly dermatological examination revealed the involvement of trunk, limbs, scalp, face and neck in the form of multiple, polysized, skin colored to erythematous, shiny, discrete nodules and papules with soft to firm consistency and waxy smooth feel, measuring 2 mm to 1 cm. Mucosae, palms and soles were spared. Excision biopsy of a nodule from the anterior abdominal wall was done and was sent for histopathological examination and immunohistochemistry (IHC). Hematoxylin and eosin stained section revealed: diffuse sheets of round to oval atypical cells with eosinophilic cytoplasm, infiltrating into the stroma of the dermis [Figure 3], with central to the eccentrically placed nucleus and coarsely dispersed chromatin with prominent centrally to peripherally placed nucleoli [Figure 4]. These cells seemed to infiltrate into the adjoining normal stroma. The tissue was further subjected to IHC, which was negative for CD3, CD20 and CD45 but positive for CD38 [Figure 5] and CD138 [Figure 6] with kappa restriction [Figure 7], showing a monoclonal origin of the plasma cells. Based on clinical, HPE and IHC findings, a final diagnosis of secondary cutaneous plasmacytoma in a known case of multiple myeloma was arrived at. Patient succumbed to her illness, 2 months after the appearance of skin lesions.
|Figure 1: Multiple hyperpigmented to violaceous papules and nodules over extremities and trunk|
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|Figure 3: Collection of cells in a nodular arrangement infiltrating into the stroma of the dermis (H and E, ×10)|
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|Figure 4: Diffuse sheets of round to oval atypical cells with eosinophilic cytoplasm, central to eccentrically placed nucleus with coarsely dispersed chromatin with prominent centrally to peripherally placed nucleoli (H and E, ×100)|
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| Discussion|| |
Multiple myeloma is a plasma cell neoplasia involving bone marrow and other tissues. It is commonly seen in fifth to sixth decade, characterized by bone pain, renal insufficiency, hypercalcemia, and infections. Various cutaneous lesions secondary to multiple myeloma have been described. They include-plasmacytomas, scleromyxedema, xanthomas, hyperkeratotic spicules, amyloidosis, pyoderma gangrenosum such as lesions, and Sweet's syndrome. Abnormal plasma cell proliferation of plasma cells outside the marrow cavity is known as extra-medullary plasmacytomas. Extramedullary plasmacytomas are further classified as either primary or secondary. Primary cutaneous plasmacytomas occur without any evidence of the simultaneous involvement of bone marrow or soft tissues and generally do not progress to myeloma. Primary cutaneous plasmacytoma is now classified under B-cell lymphoma according to World Health Organization/European Organization for Research and Treatment of Cancers. Secondary cutaneous plasmacytomas arise either by direct spread from underlying soft tissue/bony tumor or by hematogenous dissemination or rarely may arise secondary to leukemia. They develop in a small number of multiple myeloma patients, the appearance of which heralds a grave outcome. Only 20 cases of secondary cutaneous plasmacytoma are reported in literature until date. Cutaneous plasmacytomas present as violaceous or red colored papules or nodules measuring 1–5 cm in diameter, as seen in our case too.
Histopathologically, cutaneous plasmacytomas are divided into two types namely, nodular and diffuse  (our patient had nodular histology). The individual cells are pleomorphic, with atypical nuclei and prominent nucleoli, with eosinophilic cytoplasm. There may be bi- or multi-nucleate cells with mitotic figures. Both intranuclear (Dutcher's bodies) or intracytoplasmic (Russell's bodies) inclusion bodies may be found. IHC is positive for CD38, CD138, but negative for CD20 and CD45. In our case, IHC was κ-restricted, which proved a monoclonal origin of the plasma cells. Nonoptimal expression of LFA-1 and ICAM-1 in abnormal cells in plasma cell disorders has been found in patients with IgG κ type skin lesions.
Our patient's skin was involved after 2½ years of detection of multiple myeloma, which was followed by rapid progression of course of disease, leading to fatality.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]