Primary intestinal lymphangiectasia with lymphedema of lower extremities

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.