Hereditary Lymphedema

National Organization for Rare Disorders, Inc.

It is possible that the main title of the report Hereditary Lymphedema is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.


  • primary lymphedema

Disorder Subdivisions

  • None

General Discussion

Hereditary lymphedema is a genetic developmental disorder affecting the lymphatic system. It is characterized by swelling (edema) of certain parts of the body. The lymphatic system is a circulatory network of vessels, ducts, and nodes that filter and distribute certain protein-rich fluid (lymph) and blood cells throughout the body. In hereditary lymphedema, lymphatic fluid collects in the subcutaneous tissues under the epidermis due to obstruction, malformation, or underdevelopment (hypoplasia) of various lymphatic vessels. There are three forms of hereditary lymphedema: congenital hereditary lymphedema or Milroy disease; lymphedema praecox or Meige disease; and lymphedema tarda. Symptoms include swelling (lymphedema) and thickening and hardening of the skin in affected areas. In most cases, hereditary lymphedema is inherited as an autosomal dominant trait.

Lymphedema may be classified as primary or secondary. Hereditary lymphedema is also known as primary lymphedema. Secondary lymphedema occurs because of damage to the lymphatic system from surgery, radiation therapy, trauma or infection (e.g. filariasis).

Lipedema is a symmetrical accumulation of subcutaneous fat, most often in the legs. Lipedema occurs almost exclusively in females. Tenderness and bruising are alsocommon. Lipedema is frequently misdiagnosed as lymphedema.


National Lymphedema Network
116 New Montgomery Street
Suite 235
San Francisco, CA 94105
Tel: (415)908-3681
Fax: (415)908-3813
Tel: (800)541-3259

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
Tel: (914)997-4488
Fax: (914)997-4763

NIH/National Heart, Lung and Blood Institute
P.O. Box 30105
Bethesda, MD 20892-0105
Tel: (301)592-8573
Fax: (301)251-1223

Lymphovenous Canada
8 Silver Ave
Ontario, M6R 1X8
Tel: 4165332428

Vascular Disease Foundation
550 M Ritchie Highway
Severna Park, MD 21146
Tel: (443)261-5564
Tel: (888)833-4463

Lymphatic Research Foundation
261 Madison Avenue
New York, NY 10016
Tel: (516)625-9675
Fax: (516)625-9410

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site or email

Last Updated:  6/28/2012
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