Pleural effusion associated with primary lymphedema: a perspective on the yellow nail syndrome

A 28-year-old woman with bilateral pleural effusions and generalized, primary lymphedema beginning with facial erysipelas at 6 years of age is presented. The pleural effusions were exudates with 250 cells per mm3, 92 per cent of which were lymphocytes. Lymphatic stasis was demonstrated by persistence of the blue dye in the dorsa of her feet 3 months after a lymphangiogram of both lower extremities, pelvis, and abdomen. Her nails were not remarkable. Our patient represents the twentieth recorded case of pleural effusion in association with primary lymphedema. Women have been afflicted more than twice as often as men, and the age of onset has varied from birth to the eighth decade. Yellow dystrophic nails may precede or follow lymphedema or the pleural effusion and have occurred in only 11 of the 20 patients.