2016 Volume 19 Issue 2 Pages 65-70
This case involved a 6-year-old girl. Her fingers became purple in the winter a year before admission. The finger discoloration improved, but the finger swelling continued. Hardening of the skin was observed on her abdomen and upper arms three months before admission. One month before admission, she exhibited mild dyspnea when going up a hill. A collagen disease was suspected because of these symptoms and high anti nuclear antibody titer by her family doctor. She was then admitted to our hospital.
On admission, she had a high titer of anti Scl-70 antibody and ground glass opacity in the lung near the pleura on chest high resolution CT (HRCT), indicating interstitial lung disease. We diagnosed systemic sclerosis because she also had generalized skin hardening and a digital ulcer on her big toe.
We used steroid pulse therapy followed by cyclophosphamide pulse therapy for interstitial lung disease and generalized skin hardening. We also used Ca blockers and bosentan for the digital ulcer and Raynaud’s phenomenon. The digital ulcer markedly improved after starting bosentan treatment. Juvenile systemic sclerosis is very rare, and we report here her clinical course with a review of the literature.
