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May 1983

Hereditary Late-Onset Lymphedema With Pleural Effusion and Laryngeal Edema

Author Affiliations

From the Division of Pulmonary Diseases, the Departments of Medicine (Dr Herbert) and Pediatrics (Dr Bowen), University of Alberta, Edmonton.

Arch Intern Med. 1983;143(5):913-915. doi:10.1001/archinte.1983.00350050071014

• 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)