Hereditary late-onset lymphedema with pleural effusion and laryngeal edema.
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.
D000328:Adult; D000368:Aged; D005260:Female; D005540:Forced Expiratory Flow Rates; D006801:Humans; D007819:Laryngeal Edema / Q000150:complications* / Q000503:physiopathology; D008209:Lymphedema / Q000150:complications / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D010996:Pleural Effusion / Q000150:complications* / Q000503:physiopathology; D012907:Smoking; D014797:Vital Capacity