Partial lipodystrophy and dermatomyositis.

In 1978, an 11-year-old boy began to notice proximal weakness in his limbs together with facial erythema; palpebral violaceous edema; and erythematous papules on his elbows, knees, and over the metacarpophalangeal joints. A diagnosis of polymyositis/dermatomyositis was made. He was treated with oral prednisone, which caused the symptoms to disappear promptly. In 1980, proximal weakness, dysphagia, clyspnea, and similar cutaneous lesions once again developed. Muscular wasting and slight articular retractions were evident. Laboratory values at that time showed the following values or results: aspartate aminotransferase, 122 U/L (normal, 0 to 40 U/L); alanine aminotransferase, 140 U/L (normal, 0 to 40 U/L); creatine phosphokinase, 303 U/L (normal, 0 to 200 U/L); aldolase, 2.5 U/L (normal, 0.8 to 1.9 U/L); lactic dehydrogenase, greater than 1500 U/L (normal, 230 to 460 U/L); creatinine clearance, 55 mL/min (normal, 88 to 120 mL/min); antinuclear antibodies, positive at 1/320 with a homogeneous pattern; anti-DNA, anti—Sjögren syndrome A, anti—Sjögren syndrome B, and anti—ribonucleoprotein antibodies, negative; C3 and C4, normal; CH50, normal; circulating immune complexes, not detectable by the Raji cell test; and serology to Toxoplasma, negative.