In EOS SSc skin, increased EGFL7 expression may represent a homeostatic mechanism evolving to counteract the SSc-associated vasculopathy, whereas decreased expression in LSS dcSSc may suggest that after an early activation during the onset of the disease, progressive down-regulation may contribute to more severe vasculopathy such as that observed in LSS dcSSc (Fig. 1). The gene discussed is EGFL7; the disease is Leigh syndrome.