PIL may occur as:
(1) part of a systemic disorder of lymphatics (generalized)
(2) a condition limited to the intestines (localized)
Features of Primary Intestinal Lymphangiectasia (PIL):
(1) dilated lymphatics in the walls of the intestinal and/or mesentery
(2) protein-losing enteropathy with diarrhea
(3) immunodeficiency secondary to loss of both lymphocytes and immunoglobulins
(4) exclusion of secondary causes of intestinal lymphangiectasia
The lymphangiectasia may be seen in the lamina propria and submucosa, and so can be seen in intestinal biopsies of affected segments.
Complications may include:
(1) diffuse cutaneous warts (secondary to immunodeficiency)
(2) intestinal lymphoma (secondary to immunodeficiency)
(3) chylous ascites and/or pleural effusions
(4) protein malnutrition with or without peripheral edema
