PROGRESSION OF CHILDHOOD LINEAR SCLERODERMA TO FATAL SYSTEMIC-SCLEROSIS

An 8-year-old girl presented with linear scleroderma, no evidence of s ystemic disease, and a negative antinuclear antibody (ANA) test. Over the next 12 months, she functioned normally. However, over the subsequ ent 5 months, she developed dyspnea, progressive pulmonary hypertensio n, a positive ANA test, and died 17 months after presentation. At auto psy, diffuse pulmonary interstitial fibrosis, small pulmonary arterial fibroplasia, tricuspid and mitral valve subendocardial fibrosis; and distal esophageal fibrosis were seen. Contrary to suggestions in the r heumatology literature, childhood linear scleroderma, even when ANA ne gative at presentation, may progress to fatal systemic sclerosis.