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Lymphedema and Yellow Nail Syndrome

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Yellow Nail Syndrome


An expression of primary lymphedema associated with yellowish coloration of the finger nails and pleural effusions. This syndrome is also caused by same the FoxC2 gene that is responsible to what we think of as regular hereditary lymphedema.


Yellow nail syndrome is a very rare disorder often associated with lymphedema of the lower extremeties. It is also associated with lung disorders. Other associated indications are rhinosinusitis, pleural effusions, bronchiectasis.

The syndrome is characterized by yellow nails that lack a cuticle, grow slowly and are loose or detached. The nails also become dystrophic with longitudinal or transverse ridging.

There is no treatment or cure specifically for the resolution of the condition.



Gene map locus 16q24.3


A number sign (#) is used with this entry because yellow nail syndrome, included in the classification of dominantly inherited lymphedema (see 153200), can be caused by mutation in the forkhead family transcription factor gene MFH1 (FOXC2; 602402).


Samman and White (1964) delineated the yellow nail syndrome, reporting on 13 cases. The nails are typically slow growing and excessively curved, with a yellowish discoloration. They frequently show ridging due to interrupted growth. Onycholysis can occur in one or more nails.

Wells (1966) described a family with 8 cases in 4 sibships of 2 generations. In the proband, who had yellow nails, lymphedema began in the legs at the age of 51. At times edema also affected the genitalia, hands, face, and vocal cords. Lymphangiograms were interpreted as showing primary hypoplasia of lymphatics. Zerfas and Wallace (1966) described a sporadic case with onset of lymphedema at age 10. Recurrent pleural effusion occurred in some cases. 30 MEDLINE Neighbors

Govaert et al. (1992) reported a girl who was born at 33 weeks' gestation with nonimmune hydrops and a recurrent left chylothorax to a mother with the yellow nail syndrome. The nonimmune hydrops in this case was diagnosed on a 29-week ultrasound examination. Slee et al. (2000) reported a case of a newborn infant who, at 23 weeks' gestation, was found to have hydrops on antenatal ultrasonography; bilateral chylothorax was found at delivery. The mother had the yellow nail syndrome, with typical nail changes, and bronchiectasis. The infant had a recurrent cough, possibly preceding early onset of bronchiectasis. 30 MEDLINE Neighbors


Finegold et al. (2001) found a mutation in the FOXC2 gene (602402.0007) in a family with Meige lymphedema (153200), and also in a family with yellow nail syndrome. The authors observed 4 overlapped phenotypically defined lymphedema syndromes: Meige lymphedema, lymphedema-distichiasis syndrome (153400), lymphedema and ptosis (153000), and yellow nail syndrome, but not Milroy disease (153100). The authors stated that the phenotypic classification of autosomal dominant lymphedema does not appear to reflect the underlying genetic causation of these disorders. 30 MEDLINE Neighbors


1. Finegold, D. N.; Kimak, M. A.; Lawrence, E. C.; Levinson, K. L.; Cherniske, E. M.; Pober, B. R.; Dunlap, J. W.; Ferrell, R. E. :
Truncating mutations in FOXC2 cause multiple lymphedema syndromes. Hum. Molec. Genet. 10: 1185-1189, 2001.
PubMed ID : 11371511
2. Govaert, P.; Leroy, J. G.; Pauwels, R.; Vanhaesebrouck, P.; De Praeter, C.; Van Kets, H.; Goeteyn, M. :
Perinatal manifestations of maternal yellow nail syndrome. Pediatrics. 89: 1016-1018, 1992.
PubMed ID : 1594340
3. Samman, P. D.; White, W. F. :
The 'yellow nail' syndrome. Brit. J. Derm. 76: 153-157, 1964.
PubMed ID : 14140738
4. Slee, J.; Nelson, J.; Dickinson, J.; Kendall, P.; Halbert, A. :
Yellow nail syndrome presenting as non-immune hydrops: second case report. Am. J. Med. Genet. 93: 1-4, 2000.
PubMed ID : 10861674
5. Wells, G. C. :
Yellow nail syndrome with familial primary hypoplasia of lymphatics, manifest late in life. Proc. Roy. Soc. Med. 59: 447 only, 1966.
PubMed ID : 5933133
6. Zerfas, A. J.; Wallace, H. J. :
Yellow nail syndrome with bilateral bronchiectasis. Proc. Roy. Soc. Med. 59: 448 only, 1966.


George E. Tiller - updated : 10/22/2001
Victor A. McKusick - updated : 7/10/2000


Victor A. McKusick : 6/2/1986


Yellow Nail Syndrome br /


Yellow Nail Syndrome


Yellow Nail Syndrome


Yellow Nail Syndrome

GP Notebook


Multiple Effusions and Lymphedema in the Yellow Nail Syndrome

Martin Riedel, MD

From Deutsches Herzzentrum, Technische Universität München, Munich, Germany.

Correspondence to Martin Riedel, MD, Deutsches Herzzentrum, Technische Universität München, Lazarettstrasse 36, D-80636 Munich, Germany. E-mail

A 54-year-old woman was referred for evaluation of possible cor pulmonale based on the presence of dyspnea, chronic productive cough, and bilateral leg edema for 8 years. For about 8 years, her nails had been thick, brown-yellowish and would break easily. The nail grew very slowly and sometimes separated from its bed. The appearance of the nails did not change after 7 courses of antimycotic therapy. Physical examination was remarkable for dullness and decreased breath sounds at both lung bases. There was slight symmetric, nonpitting pretibial, ankle, and hand edema (Figure 1). All nails were brown-yellowish, thickened, excessively curved from side-to-side, and had transverse ridging. The lunulae were absent, and there was a distinct hump on the nails (Figure 1). Standard hematological and biochemical tests showed values within the normal ranges. Sinus radiographs revealed shadowing of both maxillary sinuses. The echocardiogram showed a small pericardial effusion (8 mm); there was no sign of constrictive pericarditis or pulmonary hypertension. The chest radiograph revealed bilateral pleural effusions with normal heart and pulmonary vasculature (Figure 2, top). Computed tomography demonstrated normal heart, great vessels and mediastinal structures, and bilateral pleural effusions. There were discrete changes suggestive of bronchiectasis in the left lower lobe. Thoracentesis revealed a clear, straw-colored nonviscous fluid with a protein content of 43.7 g/L. The concentration of glucose, lactic dehydrogenase, and amylase was normal. The white cell count was 5400/mm3 with a predominance of lymphocytes. No malignant cells were found and the culture was negative. The common causes of a transudate (cardiac failure, hepatic cirrhosis, nephropathy, myxedema, or hypoproteinemia) or exudate (lymphoma, metastatic disease, connective tissue disease, infection) were excluded. A diagnosis of pleural effusions secondary to yellow nail syndrome was made. No therapy was prescribed. A control chest radiograph after 7 weeks showed spontaneous reduction of both pleural effusions (Figure 2, bottom). Since the initial presentation the patient has been followed for 8 months and did not require thoracentesis. Chest radiographs have shown stable small pleural effusions.

The yellow nail syndrome is a triad of slow-growing dystrophic yellow nails, lymphedema, and pleural effusions, often associated with pericardial effusion, rhinosinusitis, and bronchiectasis. Our patient presented with most of these signs coexisting simultaneously. The etiology of this syndrome is obscure, although the pathogenesis seems to involve impaired lymphatic drainage. There is no known specific treatment. The pleural fluid often recurs after tapping; pleurodesis is sometimes helpful. This case illustrates the rather benign course of the syndrome over more than 8 years. The entity should be considered in the differential diagnosis of bilateral pleural effusions.


The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.

Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke's Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030;105/3/e25


Pleural effusion and recurrent broncho-pneumonia with lymphedema, yellow nails and protein-losing enteropathy.

Battaglia A, di Ricco G, Mariani G, Giuntini C.

A case is reported in whom the triad generalized lymphedema, nail dystrophy, and pleural effusion was associated to protein-losing enteropathy. This combination, not previously described, was also characterized by exacerbations of pleural effusion with recurrent episodes of broncho-pneumonia. Albumin turnover study showed depletion of the total body pool, decreased catabolic rate, and elevated albumin removal through the gastrointestinal tract. During bronchopneumonia, increased capillary permeability due to pleural involvement may worsen the basic deficit of pleural lymphatic drainage.

Publication Types:

PMID: 3979479 [PubMed - indexed for MEDLINE]


Yellow nail syndrome: complete triad with pleural protein turnover studies.

Mambretti-Zumwalt J, Seidman JM, Higano N.

Lymphedema, pleural effusions, and yellow nails constitute the yellow nail syndrome, which commonly is associated with chronic lower respiratory infections and sinusitis. Of 50 cases reported, only 13 have had the complete triad; we report another. The patient has a history of bronchiectasis, bilateral leg lymphedema, and yellow nails since 1952, with recurrent effusions since 1953. She has other conditions which are reported in high frequency with the syndrome, including sinusitis, thyroiditis, and malignancy. An albumin turnover rate was determined on the pleural effusion by a radioactive tracer method.

Publication Types:

PMID: 7403938 [PubMed - indexed for MEDLINE]


Yellow nail syndrome--the triad of yellow nails, lymphedema and pleural effusions. A review of the literature and a case report.

Nordkild P, Kromann-Andersen H, Struve-Christensen E.

The yellow nail syndrome, combination of yellow discoloured nails, lymphedema and pleural effusions, is a rare clinical condition. A review of the literature, including 97 patients, is presented. Most patients developed yellow nail syndrome in early middle age, and the overall male:female ratio was 1.1.6. The etiology of the syndrome is obscure, while the pathogenesis seems to involve impaired lymphatic drainage. A patient, whose recurrent pleural effusions were effectively controlled by chemical pleurodesis, is also presented.


Chylous ascites, intestinal lymphangiectasia and the 'yellow-nail' syndrome.

Duhra PM, Quigley EM, Marsh MN.

In 1964 Samman and White described 13 patients with lymphoedema of the lower extremities associated with an unusual dystrophy of the finger and toe nails: this they termed the 'yellow-nail' syndrome. Affected nails were thickened, excessively curved along both axes, very slow growing and of yellowish-grey hue; cuticle and lunula were usually absent and onycholysis was frequently evident. Lower limb lymphangiography in most individuals revealed hypoplasia, or aplasia of the lymphatics, similar to that occurring in primary lymphoedema: other patients also developed pleural effusions of high protein content or ascites suggestive of a more generalised disorder of the lymphatic system. Here we describe a patient in whom the classical 'yellow-nail' syndrome was associated with intestinal and chylous ascites.


Yellow nail syndrome: does protein leakage play a role?

D'Alessandro A, Muzi G, Monaco A, Filiberto S, Barboni A, Abbritti G.

Dipartimento di Medicina Clinica E Sperimentale, Universita degli studi di Perugia, Italy.

Yellow nail syndrome is characterized by primary lymphoedema, recurrent pleural effusion and yellow discoloration of the nails. Although mechanical lymphatic obstruction is assumed to be the underlying pathology, it cannot explain the common finding of high albumin concentration in the pleural space. This paper describes a case of yellow nail syndrome presenting with the classical triad of lymphoedema, recurrent pleural effusion and yellow discoloration of the nails, associated with persistent hypoalbuminaemia and increased enteric loss of albumin. Based on the findings in this case and those in the literature, it is speculated that increased microvascular permeability may contribute to the pathogenesis of this syndrome.


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