Chronic Leg Ulceration as the Presenting Feature of Diffuse Systemic Sclerosis in Childhood

A 15-year-old girl presented with a 3-year history of persistent bilateral leg ulcers. She described episodes of the Raynaud phenomenon, with severe pain, numbness, and cyanosis in her fingers and toes when exposed to the cold. Physical examination revealed a young female with thin lips, sclerodactyly, and digital infarcts on multiple fingertips. A 2-cm ulcer was seen along her left lateral malleolus and a 0.5-cm ulcer was noted proximal to the nailbed of her left great toe (Figure 1). There was additional evidence of scarring along the medial portion of her right ankle. Nailfold capillaroscopy revealed capillary dilation, tortuosity, and vessel dropout. Laboratory evaluation revealed a positive antinuclear antigen of 1:320 (homogenous pattern) and a strongly positive anti-scl-70 antibody greater than 8 U/mL, confirming the diagnosis of diffuse systemic sclerosis.

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• Figure 1 

  Chronic skin ulceration with skin erythema, induration, and hyperpigmentation.