A 58-year-old man presented to the rheumatology clinic for evaluation of skin induration of 6 months duration.
History of present illnessOne month prior to rheumatology evaluation he was admitted to the hospital for progressive worsening of shortness of breath on exertion, epistaxis, and gingival bleeding. He had fatigue, poor appetite, and an unintentional weight loss of approximately 30 pounds over the preceding 2 years. He denied symptoms of chest pain, orthopnea, paroxysmal nocturnal dyspnea, or leg swelling. Laboratory tests during that admission showed profound leukopenia of 2.2 9 10 3 /mm 3 (normal range 3.8-9.8), macrocytic anemia with hemoglobin of 5.9 gm/dl (normal range 13.8-17.2), hematocrit of 17.3% (normal range 40.7-50.3), mean corpuscular volume (MCV) of 107.7 fl (normal range 80-100), and thrombocytopenia of 3 9 10 3 /mm 3 (normal range 140-440). He underwent bone marrow biopsy, which showed aplastic anemia. He received packed red blood cell and platelet transfusions with improvement of hemoglobin and hematocrit to 7.3 gm/dl and 21.6%, respectively, and platelet counts to 32 9 10 3 /mm 3 . He was discharged to followup with hematology.In the last 6 months, he had noticed induration of the skin over his chest, abdomen, low back, arms, and thighs, and stiffness in the upper and lower extremities because of the skin induration. These areas were not painful or itchy. He had difficulty raising his arms above the level of his shoulders due to the skin changes.
Laboratory, histopathology, and radiologic evaluationAt the time of his rheumatologic evaluation, laboratory results were significant for a macrocytic anemia with