Chronic Leg Ulceration as the Presenting Feature of Diffuse Systemic Sclerosis in Childhood
Article Outline
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.
Figure 1
Chronic skin ulceration with skin erythema, induration, and hyperpigmentation.
Vasodilator treatment was initiated with sustained-release nifedipine. The patient was prescribed hydrogel dressings and an Unna boot, which resulted in dramatic improvement in leg ulceration. Further work-up revealed evidence of early pulmonary involvement as demonstrated by decreased forced vital capacity, bronchiectasis and parenchymal changes on imaging, and active alveolitis on bronchoalveolar lavage. She was started on monthly intravenous pulse cyclophosphamide, and bosentan (an endothelin inhibitor) was subsequently added to her treatment regimen. Within 6 months of initial presentation, the leg and toe ulcers had completely resolved (Figure 2).
Figure 2
Complete resolution of skin ulcer, with minimal residual scarring.
Diffuse systemic sclerosis is rare in childhood. The onset of disease is typically insidious and is characterized by the development of the Raynaud phenomenon and skin manifestations, including edema, tightening, thinning, and atrophy.1, 2 To our knowledge, this is the first report of an unusual presentation of diffuse systemic sclerosis in a young patient with chronic leg ulceration. Treatment with vasodilators, topical hydrogel dressings, and an Unna boot led to complete resolution of the leg ulcers.
References
- . Systemic sclerosis and localized scleroderma in childhood. Rheum Dis Clin North Am. 2008;34:239–255ix
- The Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for juvenile systemic sclerosis. Arthritis Rheum. 2007;57:203–212
PII: S0022-3476(11)00561-0
doi:10.1016/j.jpeds.2011.05.046
© 2011 Mosby, Inc. All rights reserved.
