The nails are markedly thickened with yellow to yellow-green discoloration of the nails. They grow slowly, at a rate of 0.25 mm/week or less. The nails may have ridges and increased side-to-side curvature, reduction of the white crescent and detachment of the nail from the nailbed. These nail abnormalities may also change over time. Most people with yellow nail syndrome have lymphedema; it is symmetrical and typically affects both legs. It is the first symptom of the condition in about a third. Involvement of the arms and face is more unusual, as is lymphedema of the abdomen with ascites and fluid collection around the heart. Various lung problems can occur in people with yellow nail syndrome. Many experience cough and shortness of breath. Forty percent of cases develop pleural effusions, which are collections of fluid in the pleural cavity. About half of all people with yellow nail syndrome have either recurrent chest infections or a chronic lung condition known as bronchiectasis which causes chronic production of sputum with episodes of worsening. Forty percent of people with yellow nail syndrome have chronic sinusitis. Yellow nail syndrome has been associated with some drugs, e.g.penicillamine, bucillamine and gold sodium thiomalate. It has also been associated with exposure to titanium from dental implants.
Genetics
Although it has been described in families, it has been suggested that it might not have a genetic link.
Diagnosis
The diagnosis is based on the combination of the symptoms. Generally, people are diagnosed with yellow nail syndrome if they have two or three of the three classical symptoms. The nail changes are considered essential for the diagnosis, but they can be subtle. Pulmonary function testing can show obstruction of the airways. People with pleural effusions may show evidence of restriction in lung volumes due to the fluid. Analysis of the fluid in pleural effusions generally shows high levels of protein but low levels of cholesterol and lactate dehydrogenase, but about 30% of effusions are chylous in that they have the characteristics of lymph. A lymphogram may be performed in people with lymphedema. This can show both under developed lymphatic ducts and dilated ducts. Dye may be found in the skin months after the initial test. Scintigraphy of lymph flow shows delays in drainage of lymph, although this test can also be normal.
People with yellow nail syndrome have been found to have a moderately reduced lifespan compared to people without the condition.
Epidemiology
The condition is thought to be rare, with approximately 150 cases described in the medical literature.
Patient Registry
Research is needed to determine the cause of YNS, to identify biomarkers for diagnosis, prognosis and response to therapy, and to find effective treatments. Patients, families, and caregivers are encouraged to join the . This is a privacy protected site that provides up-to-date information for individuals interested in the latest scientific news, trials, and treatments related to rare lung diseases.
History
The condition was first described in 1964 by London physicians Peter Samman and William White. Earlier cases may have been recorded in 1927 and 1962.
