Lymphedema, Pleural Effusions and Yellow Nails

Two siblings with congenital lymphedema had recurrent pleural effusions in adult life. The brother also had yellow toenails and thus represents an example of the syndrome of lymphedema, pleural effusions, and yellow nails. An immunologic deficiency state in these patients was suggested by recurrent bronchopthonary and skin infections, hypogammaglobulinemia, and episodes of lymphopenia. The sister developed Hodgki's disease terminally, an interesting occurrence in keeping with the increased incidence of lymphatic malignancies in patients with immunologic deficiency.