Using three experimental models, namely skin biopsy samples of SSc patients, cultured human dermal microvascular endothelial cells (HDMECs), and a BLM-induced mice SSc model of skin fibrosis, Ikawa et al. [52] showed that the development of SSc vasculopathy is associated with increased expression of endothelial CCR6 due to Fli1 deficiency in dermal small vessels irrespective of disease subtypes and disease duration, while CCL20 expression was significantly elevated in dermal fibroblasts of patients with early dcSSc. This evidence concerns the gene CCR6 and systemic sclerosis.