• 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.
(Arch Intern Med 1983;143:913-915)
Herbert FA, Bowen PA. Hereditary Late-Onset Lymphedema With Pleural Effusion and Laryngeal Edema. Arch Intern Med. 1983;143(5):913-915. doi:10.1001/archinte.1983.00350050071014