It is suspected that primary lymphedema and PLE in our patient shares the same genetic etiology of congenital dysplasia of lymphatics.[6] Gonzalez-Garay et al[11] reported that the proband with an early inactivating mutation in CELSR1 displayed lymphatic backflow and tortuous lymphatic vessels, which indicates a valvular defect of lymph propulsion in collecting vessels. This evidence concerns the gene CELSR1 and dysplasia.