Systemic Scleroderma in Childhood

Park, Wook Hwa; Park, Tae Hyun; Park, Kyung Deuk; Bang, Dong Sik; Chun, Soo Il

Abstract

The onset of systemic scleroderma in the first. decade of lif occurs in less than 1.5% of instances. Systemic sclerodrma in childhood have been characterized by less severe visceral involvement and a benign course We report a boy, aged 7 years, with a history of Raynaid; phenomenon and dysphagia. He had a mask-like face, flexion contracture of hands and knee joints, digital pitting scars, ichthyosiform skin change on leg and hyperpigrnented induration on all extremities and trunk. Antinuclear antibodies were positive in nucleolar type and Scl-70 antiocy was also present. On radiologie study, the pattern of reflux esophagitis and resorption of the listal ends of all phalanges were nated. Histopathological findings were compatible with sclerodma. He was treated with Penicillamine-D and oral corticosteroic with good improvernent.