Lymphedema and Yellow Nail Syndrome
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Yellow Nail Syndrome
http://www.lymphedemapeople.com/wiki/doku.php?id=yellow_nail_syndrome
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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
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.
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LYMPHEDEMA AND YELLOW NAILS
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.
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.
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.
George E.
Tiller - updated : 10/22/2001
Victor A. McKusick - updated : 7/10/2000
Victor A. McKusick : 6/2/1986
http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?cmd=entry&id=153300
..........................................
Yellow Nail Syndrome
http://www.diet-and-health.net/Diseases/YellowNailSyndrome.html
br /
..........................................
Yellow Nail
Syndrome
http://health.allrefer.com/pictures-images/yellow-nail-syndrome.html
..........................................
Yellow
Nail Syndrome
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Yellow%20Nail%20Syndrome
..........................................
Yellow Nail Syndrome
GP Notebook
http://www.gpnotebook.co.uk/simplepage.cfm?ID=-1838481388
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 m.riedel@dhm.mhn.de
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.
Footnotes
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
http://circ.ahajournals.org/cgi/content/full/circulationaha;105/3/e25
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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]
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=3979479&dopt=Abstract
..........................................
Yellow nail syndrome: complete triad with pleural protein turnover studies.PMID: 7403938 [PubMed - indexed for MEDLINE]
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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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