This 8-year-old girl had multiple fibrotic plaques on her legs and feet which interfered with range of motion. The skin lesions were associated with progressive systemic sclerosis complicated by bibasilar pulmonary fibrosis and lower esophageal dysmotility and dysphagia. Progressive systemic sclerosis is rare in childhood. Raynaud phenomenon occurs in 90% of patients and may present years before the onset of systemic disease. Lung, heart, kidney, gastrointestinal tract, and musculoskeletal system complications may be life-threatening. Most patients are ANA positive, and nearly 50% have antinucleolar antibodies.
Description
atrophic, fibrotic hypopigmented plaque with woody induration, localized ulceration, and scale
