This 45 year old woman was well until 6 years ago when she developed a papular facial rash that progressed to involve her face, nose, and ears. She complained of symptoms consistent with Raynaud phenomenon, and occasionally developed digital infarcts. During the last few years she has had increasing skin tightness and muscle weakness that interfered with writing, walking, and getting out of her car. The tightening of the skin in the perioral area was particularly severe. She complained of dysphagia and gastroesophageal reflux. During a hospitalization for a cardiac bypass procedure following a myocardial infarction 4 years ago she had a skin biopsy consistent with scleromyxedema. She did not tolerate treatment with oral d-penecillamine. Six months of treatment with oral cyclophosphamide and prednisone seemed to improve the skin slightly. She had several recent treatments with intravenous gamma globulin. Laboratory evaluation revealed an IgG lambda chain monoclonal gammopathy on serum protein electrophoresis.