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Primary Lymphedema


Primary lymphedema present in this
child since birth. ©Lymph Notes

 Primary lymphedema (PLE) is a genetic disorder in which lymphatic vessels fail to develop properly before birth. According to estimates from the US Department of Health and Human Services, PLE occurs in between 1/3,000 and 1/6,000 live births.

This implies that there are between 50,000 to 1,000,000 carriers of this gene in the United States. To learn more about the genetics of Primary Lymphedema, read the articles Genetics and Primary Lymphedema and Genetic Testing for Primary Lymphedema.

  • Congenital lymphedema, which is also known as Milroy’s syndrome, has visible swelling that can be detected on a prenatal ultrasound. In other cases it this condition is present at birth, or it can develop within the first two years of life. This form represents 10-25 percent of all PLE cases with females being affected twice as often as males. When swelling is present at birth, further diagnostic investigation should be conducted soon after birth because it could be a symptom of additional conditions such as Turner syndrome.
  • Lymphedema praecox, also known as Meige Disease, is a type of PLE that appears between puberty and 35 years of age. Approximately 75 percent of all PLE cases develop within this age span and it occurs in four times as many females as in males and a common characteristic is that only one leg is affected. With treatment to control the swelling, most mature females with PLE are able to live normal lives including bearing children. 
  • Lymphedema tarda, which is the rarest form of PLE, accounts for only 10% of all cases. Lymphedema tards does not become clinically evident until after the age of 35 years, or older, with swelling in one, or both, legs. Often the onset of this often condition is sudden and is of unknown cause. In some cases the cause may be a minor incident, such as an insect bite, that is followed by the swelling of the affected limb. Although the insect bite heals, the swelling does not go away.


Stage 2 lymphedema of the left arm after

breast cancer treatment. © Lymph Notes

Secondary lymphedema is an acquired disorder that is caused by damage to the lymphatic system. Cancer treatment is the largest single cause of this condition.

  • It has been estimated that there are approximately 3 million cases of SLE in the US. Cancer treatment is commonly considered to be the greatest single cause of SLE.
  • Additional causes are due to injuries, surgery, radiation, joint replacement, burns, scars, and obesity.

SLE usually develops near to where lymphatic structures have been removed or damaged with the most commonly SLE is diagnosed in the arms and legs; however, lymphedema also affects other body parts and these cases are named for the affected area or for the cause.


  • Head and Neck lymphedema is a build-up of lymph above the shoulders and collar bone. To learn more read Head and Neck Lymphedema Treatment.
  • Truncal lymhedema affects the upper portion of the body.
  • Lymphatic malformations are serious, and rare, conditions that require specialized treatment. For details, read Malformations of the Lymphatic Systems.
  • Malignant lymphedema is swelling caused by a rapidly growing tumor that places pressure on the lymph nodes. This pressure obstructs the flow of lymph and it is the tumor, not the lymphedema, that is malignant.
  • Filariasis is often described as a form of lymphedema; however, it is a totally different disorder that is caused by a parasitic worm and occurs in tropical countries. To learn about this condition click on the article Filariasis.

© LymphNotes.com 2013.This information does not replace the advice of a qualified health care professional.

Got a question or comment? Post in the 'What is Lymphedema?' forum.
Category: What is Lymphedema? Updated: 2013-05-11


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