Hereditary late-onset lymphedema with pleural effusion and laryngeal edema
F. A. Herbert and P. A. Bowen
We examined two middle-aged male cousins with unexplained edema of
postpubertal onset involving the upper and lower limbs, face, and larynx
and, in one of them, a persistent pleural effusion. Scintilymphangiography
detected an apparent paucity or absence of lymph nodes in the axillae and
above the inguinal ligaments, indicating a defect in the lymphatic systems.
Laryngeal edema, confirmed endoscopically, produced changes in one of them
in the flow volume loop characteristic of a variable extrathoracic
obstruction. A family study showed autosomal dominant transmission of the
disorder. The nosology of late-onset lymphedema is briefly discussed, with
particular reference to the so-called yellow nail syndrome.
