A Young Girl with Weather-Beaten, Waxy Knuckles

Abstract

A 9-year-old girl of mixed Asian and Caucasian descent was referred to pediatric dermatology to evaluate skin changes affecting her knuckles. She reported a 3-month history of intermittent “burning” pain and swelling of her hands. This occurred after hiking, a day at the county fair, and swimming outdoors. Blistering and ulceration accompanied the most recent episode. Progressive thickening of the skin, particularly over the knuckles, was noted. The patient also complained of headaches, fatigue, and decreased appetite. Her presumed diagnosis was dermatomyositis, but the results of laboratory studies, including a complete blood count, liver function tests, creatine phosphokinase and aldolase levels, anti-nuclear antibody screen, and rheumatoid factor, were all normal. Her past medical history was remarkable for short-stature and mild right-sided weakness caused by congenital micropolygyria of the left frontal and parietal lobes. She was not taking any medications or dietary supplements. There was no family history of skin disease, unexplained anemia, or hepatic dysfunction.