[1] Congenital lymphedema presents at birth.
Lymphedema praecox presents from ages 1 to 35.
Lymphedema tarda presents after age 35.
This type of lymphedema usually develops as a result of a developmental abnormality being precipitated by some insult such as trauma, illness, or physical immobility.
Compared to secondary lymphedema, primary lymphedema is more likely to involve the face, conjunctiva, and genitalia in association with any limbs involved.