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Vol 26, No 4 (2020)
Case report
Published online: 2021-03-22

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Primary intestinal lymphangiectasia with lymphedema of lower extremities

Kamil Klimas1, Katarzyna Drożdż1, Angelika Chachaj1, Andrzej Szuba1
Acta Angiologica 2020;26(4):140-143.


Primary intestinal lymphangiectasia (PIL), also known as Waldmann’s disease, is a rare disorder manifested
by the presence of dilated intestinal lymphatic ducts and leading to protein-losing enteropathy. PIL usually
presents early in childhood; however, rarely may be also diagnosed in adults. Suggestive laboratory findings
include hypoproteinemia, hypoalbuminemia, and hypogammaglobulinemia. Peripheral pitting edema due to
hypoalbuminemia is the main clinical feature. Peripheral lymphedema is a less common symptom. We present
a case of a 23-year-old woman with lymphedema of lower extremities and PIL diagnosed in childhood.

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