©2015 by Anais Brasileiros de Dermatologia

A 34-year-old man presented progressive gen-eralized hardening of the body for 3 years. Three months ago, he experienced difficulty in swallowing and extending his tongue, and several hard nodules were noted progressively on the forehead (Figure 1A). Physical examination revealed that the skin on his face and distal extremities was indurated (Figure 1B). On the trunk and proximal extremities, the skin was normal in elasticity, but the underlying muscles were indurated, and almost all the joints were restrict-ed in motion. No regional lymph nodes were palpa-ble. Laboratory examination showed no abnormality in routine tests. Serum and urine immunofixation showed a characteristic pattern of monoclonal IgG gammopathy, particularly of λ paraprotein. Some car-diac abnormalities including left ventricular outflow obstruction and right ventricular dysfunction were noted upon echocardiography. Positron emission tomography computed tomography (PET-CT) scan showed multiple areas of abnormal uptake in almost all the joints and some muscles. Bone marrow aspirate revealed active myeloid proliferation and 3 % of plas-macyte observed. Flow cytometry showed that CD38birght CD45dim/- cells occupied 6.5 % of all the nucleated cells and the expression of antigen wa