Clinical InvestigationsLymphedema, Pleural Effusions and Yellow Nails: Associated Immunologic Deficiency
Introduction
The association of hereditary lymphedema and pleural effusions was encountered in two siblings studied at the Montefiore Hospital.1 The purpose of this report is to provide additional data on the patients, both of whom have died; the sister (L.F.) with disseminated Hodgkin's disease and the brother (E.F.) with cor pulmonale. The combination of lymphedema, pleural effusion and yellow nails has evolved into a new clinical entity.2, 3 There is evidence to suggest that the syndrome may also include an immunologic deficiency state.
Section snippets
Case Reports
Patient E.F. was born in 1914 with edema of both lower extremities. The swelling extended proximally to involve the thighs during childhood, the scrotum after puberty, and finally the abdominal wall and hands. Frequent skin infections accompanied by high fever occurred as often as five times yearly, but responded to treatment with penicillin. The patient's sister (L.F.) and mother also had lower extremity edema. The mother was repeatedly ill with fever and infections and died of septicemia at
Lymphedema, Pleural Effusion, Yellow Nails Syndrome
E.F. and L.F. were the first reported cases of a concurrence of hereditary lymphedema and pleural effusion.1 One of our patients, E.F., also had yellow slowly growing toe nails, an observation not made in the original report. It is apparent that both patients represent examples of a new clinical entity produced by defective lymphatic circulation. Samman and White4 noted that a group of patients with impaired lower extremity lymphatic drainage had associated abnormalities, in the growth, color
References (14)
- et al.
The pathogenesis of immunologic deficiency diseases
Amer. J. Med.
(1965) - et al.
Pleural effusion in chronic hereditary lymphedema
Radiology
(1964) Yellow nails, lymphoedema, and pleural effusions
Thorax
(1966)- et al.
Primary lymphedema associated with yellow nails and pleural effusions
JAMA
(1968) - et al.
The “yellow nail” syndrome
Brit. J. Derm.
(1964) - et al.
Yellow nail syndrome with bilateral bronchiectasis
Proc. Roy. Soc. Med.
(1966) In discussion, yellow nail syndrome with bilateral bronchiectasis
Proc. Roy. Soc. Med.
(1966)
Cited by (45)
An autopsy case report of yellow nail syndrome coincided with primary biliary cholangitis
2021, Respiratory Medicine Case ReportsCitation Excerpt :Furthermore, lymphatic dilatation was observed in small intestine and peri-adrenal fat tissues, furthering the evidence that YNS is a systemic lymphatic disorder. YNS can be either idiopathic or associated with other diseases including autoimmune diseases such as rheumatoid arthritis (RA) [12], malignancies [13], immunodeficiency [14], titanium exposure [15] and so on. There have been cases whose onset of YNS were considered secondary to autoimmune thyroiditis [16] or Guillain-Barré syndrome [17].
Yellow Nails, Chronic Cough, and Edema
2010, American Journal of MedicineBronchiectasis in systemic diseases
1999, ChestAssociation between yellow nail syndrome and rheumatoid arthritis. Regression with chrysotherapy [4]
1997, Revue de Medecine InterneYellow nails, bronchiectasis and low circulating B cells
1994, Respiratory Medicine
Montefiore Hospital and Medical Center, Bronx, New York