Primary Lymphedema

Primary Lymphedema is a hereditary abnormality of the lymphatic system that occurs in approximately one in six thousand people. Although the cause is not well understood, these abnormalities often include hypoplasia or hyperplasia. The swelling of primary lymphedema usually starts distally (at the far end of the limb) and spreads proximally (upward toward the body).

Primary congenital lymphedema is also known as Nonne-Milroy's disease or Milroy’s syndrome is either present at birth or develops within the first two years of life. Shown here is primary lymphedema. Notice that the swelling is greatest nearer the toes.

Lymphedema praecox, also known as Meige disease, is primary lymphedema in which the symptoms begin at the time of puberty. Most cases of primary lymphedema present symptoms at this age and this condition affects mostly girls with symptoms in one lower extremity.

Lymphedema tarda is primary lymphedema that occurs in adults and affects both males and females. The onset of symptoms is sudden, with no apparent cause, and can affect one or both of the lower extremities.

Pediatric Primary Lymphedema