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Correspondence |

Yellow Nail Syndrome Chylous Pleural Effusions: Defective Lymph Valves Involved? FREE TO VIEW

Sotirios Zarogiannis, PhD; Chrissi Hatzoglou, MD; Paschalis-Adam Molyvdas, MD; Konstantinos Gourgoulianis, MD
Author and Funding Information

Medical School, University of Thessaly Larissa, Greece

Correspondence to: Sotirios Zarogiannis, PhD, Department of Physiology, School of Medicine, University of Thessaly, Mezourlo Hill, 41110, Larissa, Greece; e-mail: szarog@med.uth.gr


The authors have no conflicts of interest to disclose.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).


Chest. 2008;134(6):1353. doi:10.1378/chest.08-2021
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To the Editor:

We read with great interest the article by Maldonado and coworkers (August 2008),1 and we would like to comment on the rather frequent occurrence (nearly 32%) of chylous pleural effusions secondary to yellow nail syndrome (YNS) reported by the authors. It is widely accepted that YNS results from abnormal (anatomically or functionally) lymphatic network. Indeed, in a patient with typical YNS (yellow nails, lymphedema, chronic pleural effusions) in our hospital, jejunal biopsy results revealed intestinal lymphangiectasis that further confirm the systemic lymphatic network abnormality encountered in these patients.2

Contrary to physiologic conditions in which pleural fluid is absorbed mainly through solute-coupled liquid absorption by the mesothelium, in pleural effusions the main mechanism is lymphatic drainage through parietal pleura stomata.3 However, the partial occurrence of chylous effusions leads us to the hypothesis that in those patients there may be a defect in the intrinsic lymphatic mechanism (lymphatic smooth-muscle cells and valves) involving impaired lymph valves that permit lymph reflux in the pleural cavity in cases of chylous effusions.

Forkhead transcription factor (FOXC2) is normally expressed in lymphatic endothelium and is essential for the morphogenesis of lymphatic valves and the establishment of a pericyte-free lymphatic capillary network.4 An abnormally large proportion of skin lymphatic vessels was covered with smooth-muscle cells in individuals with lymphedema distichiasis and in mice heterozygous for FOXC2.4 Moreover, heterozygous mutations in FOXC2 in humans cause lymphedema distichiasis syndrome, although there are mutation carriers that do not suffer from lymphedema despite lymph reflux being present.5 Based on the above, we suggest that patients with YNS presenting chylothorax might have abnormalities in the intrinsic lymphatic mechanism, and mutations in FOXC2 gene could be a possible explanation.

Maldonado F, Tazelaar HD, Wang CW, et al. Yellow nail syndrome: analysis of 41 consecutive patients. Chest. 2008;134:375-381. [PubMed] [CrossRef]
 
Christu KA, Pastaka H, Papadopoulos D, et al. Yellow nail syndrome or diffuse lymphatic network disease. Acta Medica (Hradek Kralove). 2002;45:181-182
 
Gourgoulianis KI, Hatzoglou CH, Molyvdas PA. The major route for absorption of fluid from the pleural space. Lymphology. 2002;35:97-98. [PubMed]
 
Armulik A, Abramsson A, Betsholtz C. Endothelial/pericyte interactions. Circ Res. 2005;97:512-523. [PubMed]
 
Mellor RH, Brice G, Stanton AWB, et al. Mutations in FOXC2 are strongly associated with primary valve failure in veins of the lower limb. Circulation. 2007;115:1912-1920. [PubMed]
 

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References

Maldonado F, Tazelaar HD, Wang CW, et al. Yellow nail syndrome: analysis of 41 consecutive patients. Chest. 2008;134:375-381. [PubMed] [CrossRef]
 
Christu KA, Pastaka H, Papadopoulos D, et al. Yellow nail syndrome or diffuse lymphatic network disease. Acta Medica (Hradek Kralove). 2002;45:181-182
 
Gourgoulianis KI, Hatzoglou CH, Molyvdas PA. The major route for absorption of fluid from the pleural space. Lymphology. 2002;35:97-98. [PubMed]
 
Armulik A, Abramsson A, Betsholtz C. Endothelial/pericyte interactions. Circ Res. 2005;97:512-523. [PubMed]
 
Mellor RH, Brice G, Stanton AWB, et al. Mutations in FOXC2 are strongly associated with primary valve failure in veins of the lower limb. Circulation. 2007;115:1912-1920. [PubMed]
 
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