Primary hypertrophic osteoarthropathy with myelofibrosis

Primary hypertrophic osteoarthropathy or pachydermoperiostosis is a rare congenital disease characterized by clubbing of the fingers, periostitis of the distal long bones, and hypertrophic skin changes (pachydermia) including thick folds in the skin of the face, forehead, scalp and extremities, and also joint pain. Clinical manifestations of this disease generally appear among the young and middle-aged. In this article we report a case of a 43-year-old man with pachydermoperiostosis. His skin and joint manifestations were prominent. He had also anemia, and bone marrow biopsy showed myelofibrosis.