CELSR1 and lymphedema: LAs result from both germline autosomal dominant and recessive variants with varying degrees of penetrance as well as somatic variants.1 Lymphedema arises from inherited and de novo germline loss of function (LOF) variants in lymphangiogenic genes, ANGPT2, CCBE1, CELSR1, FLT4, FOXC2, GATA2, GJA1, GJC2, HGF, KIF11, IKBKG, SOX18, TIE1, PTPN14, and VEGFC.