The levels of various pro-inflammatory cytokines (interleukin [IL]‐6, IL‐1β, IL‐2R, IL‐8, tumor necrosis factor‐α [TNF‐α], and interferon [IFN]‐γ) and immune cells (monocytes, macrophages, neutrophils, dendritic cells, NK cells, CD4+, and CD8+ T cells) are elevated, resulting in excessive activation of the immune system and occurrence of inflammatory CS. The gene discussed is IL2RA; the disease is Cowden syndrome 1.