Yellow Nail Syndrome
National Organization for Rare Disorders, Inc.
Synonyms
Disorder Subdivisions
General Discussion
Yellow Nail Syndrome is a very rare disorder involving a combination of lymphedema (swelling) of the lower extremities, recurrent pneumonia, bronchiectasis, and yellowed nails. Most patients have disease of the lower lobe of the lung which may be due to obstruction and/or infection. (Bronchiectasis is an irreversible enlargement of one or more of the bronchi due to the destruction of the muscular and elastic supporting tissues.)
Symptoms
Yellow Nail Syndrome is characterized by slow growing, yellow, thickened nails with a loss of cuticles. The nails may become convex and loose (onycholysis). This condition is usually associated with plural effusion (fluid filled lungs) and/or lymphedema (fluid filled lymphatic vessels) of the extremities. Intermittent edema of the genitalia, hands, and face may also be present.
Respiratory diseases such as bronchiectasis (chronic inflammation or degenerative condition of the bronchi and bronchioles), bronchitis (chronic inflammation of the bronchial tubes) and sinusitis (inflammation of the membrane lining the sinus) may also occur with Yellow Nail Syndrome. .
Causes
The exact cause of Yellow Nail Syndrome is not known, but recent evidence suggests that it is genetic in nature and inherited as an autosomal dominant trait. Since it is often associated with respiratory infections, it may be that the immune system is involved, as well.
Human traits, including the classic genetic diseases, are the product of the interaction of two genes, one received from the father and one from the mother. In autosomal dominant disorders, a single copy of the disease gene (received from either the mother or father) will be expressed "dominating" the other normal gene and resulting in the appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is 50 percent for each pregnancy, regardless of the sex of the resulting child. The risk is the same for each pregnancy. .
Affected Populations
Women may be afflicted with this syndrome more often than men and the onset varies from birth to the eighties.
Standard Therapies
There is no known treatment for Yellow Nail Syndrome, but the nails may improve when the related disorder is treated.
References
McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore. MD: The Johns Hopkins University; Entry No: 153300; Last Update: 7/10/2000.
REVIEW ARTICLES Glazer M, et al. Successful talc slurry pleurodesis in patients with nonmalignant pleural effusion. Chest. 2000;117:1404-09.
Hershko A, et al. Yellow Nail Syndrome. Postgrad Med J. 1997;73:466-68.
JOURNAL ARTICLES Slee J, et al. Yellow nail syndrome presenting as nonimmune hydrops: second case report. Am J Med Genet. 2000;93:1-4.
Moffitt DL, et al. Yellow nail syndrome: the nail that grows half as fast grows twice as thick. Clin Exp Dermatol. 2000;25:21-23.
Widjaja A, et al. Octreotide for therapy of chylous ascites in yellow nail syndrome. Gastroenterology. 1999;116:1017-18.
Sacco O, et al. Yellow nail syndrome and bilateral cystic lung disease. Pediatr Pulmonol. 1998;26:429-33.
Paradisis M, et al. Yellow nail syndrome in infancy. J Paediatr Child Health. 1997;33:454-57.
Resources
American Lung Association
61 Broadway, 6th Floor New York, NY 10006 USA Tel: 2123158700 Fax: 2123158870 Tel: 8005864872 Internet: http://www.lungusa.org
NIH/National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse
1 AMS Circle Bethesda, MD 20892-3675 USA Tel: 3014954484 Fax: 3017186366 Tel: 8772264267 TDD: 3015652966 Email: NIAMSinfo@mail.nih.gov Internet: http://www.niams.nih.gov
NIH/National Heart, Lung and Blood Institute Information Center
P.O. Box 30105 Bethesda, MD 20824-0105 Tel: (301)592-8573 Fax: (301)251-1223 Email: nhlbiinfo@rover.nhlbi.nih.gov
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.
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Last Updated: 9/20/2000
Copyright 1990, 1999, 2000
National Organization for Rare Disorders, Inc.
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