According to currently available studies, a number of cytokines (such as IL-6, TNFα, IFNγ, and others) chemokines (such as CCL2, CXCL8, CXCL10 and others) and infiltrating cells (such as neutrophils, monocytes, t-cells, macrophages and others) were shown to be involved in the immune mechanisms sustaining CS. Here, CXCL10 is linked to Cowden syndrome 1.