Scleromyxedema is an unusual skin disease that is characterized by a proliferation of fibroblasts and an accumulation of mucin. Despite experience with multiple forms of therapy, no single agent has proved uniformly beneficial in this condition. We describe a patient with scleromyxedema who was treated successfully with 2-chlorodeoxyadenosine (2-CdA), a purine analog used mainly in the treatment of lymphoproliferative disorders.