Interestingly, CCN2 expression, like S100A9 (Figure 2), is increased in the epidermal layers of systemic sclerosis skin [61], a fibrotic disease state associated with TNIP1 SNPs in patient genomes [62] and TNIP1 protein deficiency [15] in lesional skin. This evidence concerns the gene TNIP1 and systemic sclerosis.