Primary lymphedema in the nephrotic syndrome: case report

The aim of this case report is to discuss possible connections between the development of a hypoproteinaemic oedema due to the nephrotic syndrome and the occurrence of lymphoedema. Two patients (a three year-old girl and a seven year-old boy) developed lymphoedema of one leg one year after the onset of the nephrotic syndrome. The case of the six year-old girl is presented. Malignancy was excluded by clinical investigation. Direct lymphography failed to show any peripheral lymph-vessels; indirect lymphography (i.c. infusion of a newly-developed contrast medium) revealed hypoplasia of the peripheral lymph-collectors. The development of lymphoedema 12-18 months after the appearance of the nephrotic syndrome supports the hypothesis that the increase in extravascular fluid, which is caused by a reduced oncotic pressure in the plasma, may trigger off the development of lymphoedema if there is a primary defect of the lymphatic system.