Scleroderma Associated with Idiopathic Myelofibrosis

A patient is described who developed scleroderma one and a half years: after the diagnosis of idiopathic myelofibrosis (IM). In addition to suffering from the hematological features characteristic of the osteosclerotic phase of IM, the patient also developed Raynaud's phenomenon, marked distal sclerosis in the hands and painful ulcers on the fingertips, with physical findings consistent with scleroderma and positive anti-nuclear antibodies (fine-speckled pattern). The skin lesions showed a dramatic improvement after prednisone treatment but the patient eventually died of progressive myelofibrosis. The present case adds to those previously reported on the occurrence of immunological abnormalities in patients with IM.