Primary lymphedema: Four cases with vessel and node findings

Primary lymphedema is thought to be due to a congenital or developmental defect in the lymphatic system with a delayed clinical onset in many cases because of collateral lymphatic flow. Abnormality of lymph vessels was originally considered to be solely responsible for the pathogenesis; however, nodal abnormalities have recently been described. Four cases of primary lymphedema with various degrees of lymph vessel and node involvement are presented. The lymphographic appearances are discussed, nodal findings are emphasized, and the concept of primary lymph node abnormality is supported.