Clinical History.—A 12-year-old girl was admitted to the hospital in 1972 with facial erythema, progressive weakness, and dyspnea.
Physical examination disclosed a generalized erythematous rash on the face and upper and lower extremities. There was severe proximal muscle tenderness and weakness. The rest of the physical examination showed no abnormalities.
The hematocrit value, white blood cell count, and serum electrolyte, calcium, phosphorus, creatinine, and urea nitrogen levels were normal. The creatinine phosphokinase level was 372 international units (IU) (normal, 5-30 IU). Evaluation of arterial blood gases with the patient breathing room air showed a pH of 7.43; carbon dioxide pressure, 37.5 mm Hg (Denver normal, 36 ± 2 mm Hg); and oxygen pressure, 47 mm Hg (Denver normal, 70 ± 5 mm Hg). Pulmonary function tests disclosed a severe restrictive defect with decreased vital capacity and total lung capacity. The electrocardiogram was normal. A chest roentgenogram was taken (Figure).