Yellow nail syndrome, also known as "primary lymphedema associated with yellow nails and pleural effusion", is a very rare

medical Medicine is the science and practice of caring for a patient, managing the diagnosis, prognosis, prevention, treatment, palliation of their injury or disease, and promoting their health. Medicine encompasses a variety of health care practic ...

syndrome A syndrome is a set of medical signs and symptoms which are correlated with each other and often associated with a particular disease or disorder. The word derives from the Greek language, Greek σύνδρομον, meaning "concurrence". When a sy ...

that includes

pleural effusion A pleural effusion is accumulation of excessive fluid in the pleural space, the potential space that surrounds each lung. Under normal conditions, pleural fluid is secreted by the parietal pleural capillaries at a rate of 0.6 millilitre per k ...

lymphedema Lymphedema, also known as lymphoedema and lymphatic edema, is a condition of localized swelling caused by a compromised lymphatic system. The lymphatic system functions as a critical portion of the body's immune system and returns interstitial fl ...

(due to under development of the

lymphatic vessel The lymphatic vessels (or lymph vessels or lymphatics) are thin-walled vessels (tubes), structured like blood vessels, that carry lymph. As part of the lymphatic system, lymph vessels are complementary to the cardiovascular system. Lymph ve ...

s) and yellow

dystrophic Dystrophic lakes, also known as humic lakes, are lakes that contain high amounts of humic substances and organic acids. The presence of these substances causes the water to be brown in colour and have a generally low pH of around 4.0-6.0. Due to ...

nails. Approximately 40% will also have bronchiectasis. It is also associated with chronic

sinusitis Sinusitis, also known as rhinosinusitis, is inflammation of the mucous membranes that line the sinuses resulting in symptoms that may include thick nasal mucus, a plugged nose, and facial pain. Other signs and symptoms may include fever, head ...

and persistent coughing. It usually affects adults.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. .

Signs and symptoms

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 (four fifths) 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 Ascites is the abnormal build-up of fluid in the abdomen. Technically, it is more than 25 ml of fluid in the peritoneal cavity, although volumes greater than one liter may occur. Symptoms may include increased abdominal size, increased weight, a ...

(fluid collection in the abdominal cavity) and fluid collection around the heart. Various lung problems can occur in people with yellow nail syndrome. Many experience cough and

shortness of breath Shortness of breath (SOB), also medically known as dyspnea (in AmE) or dyspnoea (in BrE), is an uncomfortable feeling of not being able to breathe well enough. The American Thoracic Society defines it as "a subjective experience of breathing di ...

. Forty percent of cases develop

s, which are collections of fluid in the

pleural cavity The pleural cavity, pleural space, or interpleural space is the potential space between the pleurae of the pleural sac that surrounds each lung. A small amount of serous pleural fluid is maintained in the pleural cavity to enable lubrication b ...

(the space that contains the lungs and normally only has a minimal amount of fluid in it). 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

. Yellow nail syndrome has been associated with some drugs, ''e.g.''

penicillamine Penicillamine, sold under the brand name of Cuprimine among others, is a medication primarily used for the treatment of Wilson's disease. It is also used for people with kidney stones who have high urine cystine levels, rheumatoid arthritis, ...

bucillamine Bucillamine is an antirheumatic agent developed from tiopronin. Activity is mediated by the two thiol In organic chemistry, a thiol (; ), or thiol derivative, is any organosulfur compound of the form , where R represents an alkyl or other org ...

and

gold sodium thiomalate Sodium aurothiomalate (INN, known in the United States as gold sodium thiomalate) is a gold compound that is used for its immunosuppressive anti-rheumatic effects. Along with an orally-administered gold salt, auranofin, it is one of only two go ...

. It has also been associated with exposure to titanium from dental implants or consuming food containing titanium dioxide.

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 (yellow nails, lymphedema and pleural effusion). The nail changes are considered essential for the diagnosis, but they can be subtle.

Pulmonary function testing Pulmonary function testing (PFT) is a complete evaluation of the respiratory system including patient history, physical examinations, and tests of pulmonary function. The primary purpose of pulmonary function testing is to identify the severity ...

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 Lactate dehydrogenase (LDH or LD) is an enzyme found in nearly all living cells. LDH catalyzes the conversion of lactate to pyruvate and back, as it converts NAD+ to NADH and back. A dehydrogenase is an enzyme that transfers a hydride from one ...

, but about 30% of effusions are chylous ( chylothorax) in that they have the characteristics of

lymph Lymph (from Latin, , meaning "water") is the fluid that flows through the lymphatic system, a system composed of lymph vessels (channels) and intervening lymph nodes whose function, like the venous system, is to return fluid from the tissues ...

. A lymphogram may be performed in people with lymphedema. This can show both under developed (hypoplastic) lymphatic ducts and dilated ducts. Dye may be found in the skin months after the initial test.

Scintigraphy Scintigraphy (from Latin ''scintilla'', "spark"), also known as a gamma scan, is a diagnostic test in nuclear medicine, where radioisotopes attached to drugs that travel to a specific organ or tissue (radiopharmaceuticals) are taken internally and ...

of lymph flow (lymphoscintigraphy) shows delays in drainage of lymph (sometimes asymmetrically), although this test can also be normal.

Treatment

Normal treatment for swelling and any respiratory problems is appropriate. Nutritional supplementation with

Vitamin E Vitamin E is a group of eight fat soluble compounds that include four tocopherols and four tocotrienols. Vitamin E deficiency, which is rare and usually due to an underlying problem with digesting dietary fat rather than from a diet low in vi ...

in some studies has been shown to be effective in controlling nail changes.

Prognosis

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.

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.

References

External links

{{DEFAULTSORT:Yellow Nail Syndrome Conditions of the skin appendages Respiratory diseases Syndromes