Primary lymphedema is hereditary and results from genetic anomalies that lead to deficient levels of proteins that regulate lymphangiogenesis and lymphatic function, including VEGFR3, FOXC2, SOX18, collagen and calcium binding EGF domains 1 (CCBE1), ADAM metallopeptidase with thrombospondin type 1 motif 3 (ADAMTS3) and GATA2 (Fang et al., 2000; Irrthum et al., 2000; Irrthum et al., 2003; Alders et al., 2009; Kazenwadel et al., 2012; Brouillard et al., 2017; Jones and Mansour, 2017; Oliver et al., 2020). This evidence concerns the gene CCBE1 and primary lymphedema.