Dermal fibroblasts explanted from skin biopsies of VEDOSS patients (n = 6), showed 3- to 7-fold increase in profibrotic gene expression (COL1A1, COL1A2, ACTA2 and CCN2) relative to HC (Fig. 2A–D), similar to SSc fibroblasts (N = 4). This evidence concerns the gene ACTA2 and systemic sclerosis.