What causes lymphedema praecox?

Updated: Mar 24, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Answer

Lymphedema praecox, also known as Meige disease, is the most common form of primary lymphedema. By definition, this disease becomes clinically evident after birth and before age 35 years. The condition accounts for 65-80% of all primary lymphedema cases and most often arises during puberty. About 70% of cases are unilateral, with the left lower extremity being involved more often than the right. Histologically, these patients are likely to demonstrate a hypoplastic pattern, with the lymphatics reduced in caliber and number.

Females are affected 4 times as often as males. The fact that lymphedema praecox usually manifests clinically around menarche suggests that estrogen may play a role in its pathogenesis.


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