Firstly, the development of the local inflammatory state in the pancreas initiates the release of many pro-inflammatory factors, i.e., growth factors, lipopolysaccharides, TGF-β, TNF-α, which activate signaling pathways in cells (TLR-4—Toll like receptor 4, mTOR, NF kappa B/MAPK) potentially promoting the development of PC. Here, TLR4 is linked to pachyonychia congenita.