Contributed by Wen-Wei Chung, MD, PhD and Robert E. Lee, MD
Published on line in May 1998
This 35 year-old white female had arthralgia since 1983, and developed Raynaud's phenomenon and myalgia later. She had been under stable control with Methotrexate and Plaquenil from 1993 to 7/1997, when she developed cardiac tamponade requiring a pericardial window. An echocardiogram revealed mild right ventricular dilatation, with tricuspid and pulmonary regurgitation. She was referred to UPMC on 9/26/1997 for a two-month history of progressive weakness and shortness of breath with dry cough. Meanwhile, she had photosensitivity, morning stiffness, superior vena cava obstruction, glossitis and eye congestion. The clinical studies disclosed myositis with increased levels of serum creatinine kinase. On admission, she had telangiectasia on her face, multiple cutaneous calcinosis in her chest (Image 01) and extremities (Image 02 and 03). She had proximal muscle weakness, decreased pulses and cyanosis in all extremities, with dry gangrene as well as atrophy involving multiple distal digits of her hands. During the hospital course, her dyspnea did not improve. She had low grade fever, diffuse coarse breathing sounds with crackles, and decreased air movement. One week after admission, she had sinus tachycardia, a septal myocardial infarct, and incomplete right bundle branch block; she then developed multifocal atrial premature contractions. A leukocytosis with increased neutrophilic differential count was noted. In spite of treatment with multiple antibiotics, steroids, cytoxan, and anticoagulants, she died on the 10th day after admission.
The chest X-ray showed extensive subcutaneous calcification involving the axilla and bilateral chest walls (Image 01). X-rays of hand and elbow revealed multifocal subcutaneous and muscle fascial calcification (Image 02 and 03).