Primary Congenital Lymphedema.

We present the case of a 26-year-old man with congenital primary lymphedema. A lymphedema of the right upper limb appeared at the age of 2 years, followed by edemas of the right lower limb and of the right part of the head and neck. A lymphedema of the left lower limb began 5 years ago, and finally an edema of the external genitalia began 2 years ago. Lymphoscintigraphy of the lower limbs and pelvic/abdominal region (including SPECT/ldCT) revealed interesting abnormalities.