hematologist Clinic of hematology, CLinical Center Sarajevo University Sarajevo, Bosnia and Herzegovina
Introduction: ABSTRACT Cutaneous manifestations of multiple myeloma are very rare with fewer than 100 cases described in the literature so far. Lesions appear in the form of nodules or plaques that can be erythematous, purple or skin-colored with a diameter of 0.5 to 3 cm. These lesions are sign of poor prognosis and refractory to standard therapy. We present the case of a 49-year-old male patient with multiple myeloma, who at the time of relapse presented with skin lesions, so far first reported case in BIH.
Methods: The diagnosis of multiple myeloma, IgG kappa in clinical stage III – R/ISS was made in May 2020 at the Clinic of Hematology of the Clinical Center of the University of Sarajevo. Until October 2021, he was treated with chemotherapy, irradiation, and autologous transplantation followed by maintenance therapy with Revlimid. In decembar 2021. the patient was admitted with painful purple skin nodules localized in the right femoral region. Biochemical findings were consistent with anemia (Hb 96), thrombocytopenia (Plt 58), renal imparement (urea 11.2, creatinine 208), increased IgG, kappa light chains and Bence-Jonce proteinuria. A punch biopsy of the nodule showed infitration of entire dermis with monoclonal plasma cells. The cells were: CD38+, CD138+, CD117 focal+, CD56 +, LCA-, MPO staining of individual cells, CK-, CD34 + in blood vessels. Numerous atypical mitoses are present. A bone marrow biopsy showed up to 5% moncolonal plasma cells. He received Revlimid/Melphalan/Dexa, one cycle. Skin lesions resolved partially. Unfortunately he deceased month later, due to the progressionth of myeloma.
Results: Cutaneous lesions of myloma tend to occur with relapses and in highly aggressive and progressive forms of multiple myeloma. In both cases the prognosis is poor with an average life expectancy of 12 months post diagnosis median survival is about 8 months, with less than 20% of patients found to be progression free at 5 years. Histopathological findings are characterised by monomorphic dermal and subcutaneous infiltrates of plasma cells. Immunohistochemically we see monoclonality of the plasma cells which have strong immunoexpression for CD138 (6). These lesions can occur anywhere in the skin but are most commonly seen in the chest and abdomen (44%) involvement of the skin of the extremities is less common.
Conclusions: Treatment for cutaneous plasmacytoma in multiple myeloma involves chemotherapy, steroids and local radiotherapy. Surgical excision has a role in lesions resistant to radiotherapy or where lesions are very large and symptomatic.