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Hereditary Lymphedema
Abstract
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NORD is very grateful to Joseph L. Feldman, MD, Senior Clinician Educator, Pritzker School of Medicine University of Chicago; Director, Lymphedema Treatment Center, NorthShore University Health System, for assistance in the preparation of this report.
Synonyms of Hereditary Lymphedema
- primary lymphedema
Disorder Subdivisions
- congenital hereditary lymphedema
- hereditary lymphedema, type I
- lymphedema-distichiasis
- lymphedema praecox
- lymphedema tarda
- Milroy disease
- Nonne-Milroy disease
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.
Organizations related to Hereditary Lymphedema
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