A parent is concerned because her 9-year-old daughter has been diagnosed with "localized scleroderma," after years of seeing physicians and not getting a diagnosis. A dermatologist told her to do nothing and to wait and see what happens. In contrast, a rheumatologist advised that her daugher be put on medications to prevent the scleroderma from getting worse. Who is right? What could be the downside of an aggressive approach?

Henry L. Wright, MD

Response from Robert Fox, MD

The term localized scleroderma requires further definition. The term might mean morphea without any manifestations of a systemic autoimmune disease, and this requires only observation.

Scleroderma also may be characterized as being made up of calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction hypermotility, sclerodactylia, and telangiectasia (CREST) and diffuse (both usually with positive antinuclear antibodies, CREST with anticentromere antibodies, and diffuse with antitopoisomerase antibody). The therapeutic intervention in these conditions is based on the presence of systemic manifestions, including myalgia (myositis), Raynaud's phenomenon, hypertension, and visceral (esophageal, lung, kidney) involvement. The information in the question suggests that the child has morphea (localized skin lesion with features similar to scleroderma). I would be initially conservative until the tempo of the disease process is clear.


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