What is Yellow Nail Syndrome?
Yellow nail syndrome (YNS) is a rare medical condition that is usually identified by the combination of two out of three key features. First, the presence of slow-growing, hard, yellow, and deformed nails. Second, lymphedema – a condition where parts of the body swell due to damaged or blocked lymphatic system. Finally, diseases of the respiratory tract, which include our airways and lungs. YNS was first reported in 1927 and it mostly affects adults over 50, but rare cases have also been reported in children and even newborns.
This syndrome can affect the fingernails, toenails, respiratory system, and parts of the body that are prone to fluid buildup due to gravity, typically the lower legs. These symptoms are believed to be caused by problems with the lymphatic drainage – a network of vessels and nodes in the body that plays a role in our immune response and removal of waste products.
As the syndrome’s name implies, a common feature of YNS is yellow nails, also referred to as xanthonychia. However, yellow nails are not always necessary for diagnosis if 2 of the other clinical signs are present. Nail discoloration can range from pale yellow to dark green, and nails can be either cloudy or clear. These symptoms can often be mistaken for a fungal nail infection, as nails may become thick and hard.
In over half of the YNS cases, the respiratory tract is involved. The most common symptom is a chronic cough, followed by the build-up of fluid in the lung lining, known as pleural effusion. Other lung-related complications include widened airways, or bronchiectasis, repetitive pneumonia, sinus infections, and lung scarring.
Lymphedema, or swelling in both lower legs, occurs in between 30% to 80% of YNS cases. The swelling associated with YNS can be easily mistaken with fluid buildup seen in patients with a failing heart. In cases of lymphedema, treatments often include massage, compression wraps, exercises, and less commonly, surgical procedures.
Diagnosing YNS can be challenging, as patients may not present all three clinical signs simultaneously. For example, swelling can be the first symptom in about a third of YNS diagnoses. The way the disease develops and prognosis largely depends on individual’s symptoms and when the diagnosis is made. In some mild cases, symptoms of YNS can go away without treatment, but in most cases symptoms persist and relapse, requiring ongoing care. YNS can significantly impact quality of life, and can lead to additional health problems such as skin infections, lung infections, and buildup of fluid in the lungs.
What Causes Yellow Nail Syndrome?
The exact cause of Yellow Nail Syndrome (YNS), a condition where nails thicken and turn yellow, is unknown. The most common belief is that it’s due to problems with the lymphatic system, especially with the draining of fluid from body tissues. Imaging tests that visualize the lymphatic system have shown abnormalities in people with YNS.
Interestingly, these tests saw a big difference in lymph drainage between patients with YNS and those with primary lymphedema, another condition due to poor lymph fluid drain.
Another theory is that exposure to titanium, specifically titanium dioxide (a substance found in dental and joint implants, surgical staples and various cosmetics) might also be a cause for YNS.
There’s evidence that nail clippings from patients with YNS contained high levels of titanium. Moreover, in some cases, when titanium implants were removed, the symptoms of YNS went away. There are also few cases reported after heart valve replacement surgery.
Although cases of inherited and present from birth YNS have been reported, most cases of YNS appear later in life and seem to be an acquired disorder related to the lymphatic system. It’s also been linked with certain types of cancer, immune system diseases, and disorders where the immune system is not working properly.
Risk Factors and Frequency for Yellow Nail Syndrome
Yellow Nail Syndrome (YNS) is a rare condition, with less than one case in a million people. Most of the current knowledge about is based on individual case reports. It’s more likely to occur in people over 50, and affects both men and women equally. It’s extremely rare in children or to be present at birth. If a young person is thought to have YNS, extra care should be taken to check for other health problems linked to the lymphatic system.
Signs and Symptoms of Yellow Nail Syndrome
Yellow Nail Syndrome (YNS) is a rare medical condition and can be diagnosed by an in-depth review of a patient’s health history, particularly relating to the respiratory, vascular, and skin systems. Doctors often pay attention to persistent cough, congestion, and recurring respiratory infections. Knowing a patient’s health history, smoking habits, exposure to respiratory irritants, and previous chest imaging also proves significant. However, it’s important to note that symptoms can be mild, come and go, and may not all appear at the same time.
In examining for YNS, careful attention should be given to the nails, even requiring the removal of nail polish. The nails can exhibit various changes such as discoloration (varying from pale yellow to green), changes in thickness or irregularities, slowness in growth, and brittleness.
YNS can also cause ‘lymphedema’, a health condition which isn’t different from primary lymphedema (swelling usually in the arms or legs). This may appear as the first symptom in about one-third of patients and between 30 to 80 percent of individuals diagnosed with YNS have it. While the reliability of physical signs can vary, one useful indicator is the Kaposi-Stemmer sign (inability to pinch and lift the skin at the base of the second toe). Initially, you might see ‘pitting’ (indentations left after you press the skin) in the lymphedema, but as the swelling advances and the skin thickens, it becomes less noticeable. It’s worth mentioning that it can be challenging to tell the difference between swelling related to vein obstruction and lymphedema.
People with YNS may often experience respiratory issues. These symptoms are found in 60-70 percent of patients, with chronic cough being the most common. Careful questioning and examination can direct this process. Doctors might identify fluid build-up inside the chest (pleural effusions) through listening to the chest sounds and tapping on the chest. Signs of chronic sinusitis, which include facial fullness, sinus tenderness, and swollen nasal tissues, might also be present.
Testing for Yellow Nail Syndrome
Diagnosing Yellow Nail Syndrome (YNS) is mostly done by a doctor observing your symptoms and no unique tests are required. However, it’s essential to ensure that other, more common conditions like heart failure, lymphedema (swelling due to blocked lymph vessels), and onychomycosis (a fungal infection of the nails) are not causing your symptoms. Careful analysis of your medical history and a thorough physical examination can help do this. Sometimes, additional tests might be necessary, these could include:
1. Echocardiography: This is a type of ultrasound test that uses sound waves to create pictures of your heart. It can be used to check if heart failure is causing your symptoms.
2. Chest X-ray: This is a common imaging test that can identify conditions like pneumonia or other lung-related problems.
3. CT scan of the sinuses: This imaging test creates detailed pictures of your sinuses and can be used to check for chronic sinusitis, a long-term inflammation of the sinuses.
4. Testing for infection: Your doctor may require you to provide a sputum (mucus) sample or have a blood test done to check for possible infections.
5. Analysis of nail scrapings or clippings: Your nails may be examined under a microscope to rule out onychomycosis.
6. Thoracentesis: In this procedure, fluid from the area around the lungs is removed and tested. This can help to identify possible causes of your symptoms.
7. Lymphoscintigraphy: This is a special type of imaging test that helps to check if the lymphatic system is working properly. If lymph vessels are blocked (lymphatic insufficiency), it could lead to symptoms like those of Yellow Nail Syndrome.
Treatment Options for Yellow Nail Syndrome
Yellow Nail Syndrome (YNS) is an unusual condition where treatment usually focuses on managing the symptoms. If YNS is thought to be related to a cancer diagnosis (a situation often described as a ‘paraneoplastic syndrome’), treating the cancer can sometimes also solve the symptoms of YNS. In some circumstances, YNS can even get better on its own without needing any treatment.
When dealing with symptoms related to your lungs, a condition known as ‘pleural effusions’ often needs to be treated. This condition happens when there is too much fluid around your lungs, which can be removed using a procedure known as ‘thoracentesis’. There’s a medication called Octreotide that has been shown to work in some cases, but unfortunately, fluid often comes back. To fix this recurring issue, other treatment options like decortication (a surgery on the lungs), pleurodesis (a procedure to stick the lung to the chest wall to prevent fluid build-up), or thoracic duct embolization (a way to stop abnormal fluid leakage) might be required. If you have recurring infections or advanced bronchiectasis, a condition where the airways in your lungs become wider, your doctor could suggest antibiotics. Vaccines are also commonly recommended, especially against pneumonia (commonly referred to as pneumococcal vaccine) and the flu (seasonal influenza vaccine).
Another key symptom of YNS is diseased and discolored nails. The conditions affecting the nails can improve on their own without any intervention. There’s mixed evidence about the effectiveness of oral vitamin E in treating nail discoloration. Despite the absence of fungal infections, treatments with antifungals, sometimes in combination with vitamin E, are often used. Other treatments like oral zinc, clarithromycin (an antibiotic), and corticosteroid injections (steroids for reducing inflammation) are also suggested, but there’s limited solid evidence to support this.
If you also experience lymphedema, which is swelling in your limbs due to a blockage in the lymphatic system, the same interventions used in primary lymphedema are applied. Usually, doctors prefer non-surgical treatments. This can involve using compression garments and special bandaging, in combination with skincare, a massage technique known as manual lymph drainage to encourage the fluid to flow away from the swollen area, and exercises.
What else can Yellow Nail Syndrome be?
Yellow Nail Syndrome (YNS) can be challenging to diagnose due to it affecting various organs and exhibiting a wide range of symptoms. Several other conditions can show similar signs and may need to be ruled out.
For example, the lung problems seen in YNS, like thickening and plaques on the pleura (linings of the lungs) bear a resemblance to the outcomes of asbestos-related lung disease.
Heart failure, which is more common than YNS, also presents with symptoms like swelling in the lower extremities and fluid buildup in the lungs. Interestingly, the swelling caused by congestion in heart failure usually reduces with diuretic drugs (water pills) – something not observed in the swelling caused by YNS.
Also, other systemic (body-wide) diseases such as arthritis-related and autoimmune conditions, various types of cancer, hormone abnormalities, and immune deficiencies may also cause yellowing of the nails, a common sign in YNS. Nail fungal infection, also known as onychomycosis, should also be ruled out in individuals presenting with yellow and disfigured nails.
What to expect with Yellow Nail Syndrome
The outcome of Yellow Nail Syndrome (YNS) is dependent on the specific symptoms of the patient and any other diseases they may have. Symptoms could be mild, such as unattractive fingernails or a persistent cough, or severe, like recurring, significant swelling or constant lung infections. Unfortunately, there is no known cure for YNS, so treatment is focused on making the symptoms more bearable.
Possible Complications When Diagnosed with Yellow Nail Syndrome
YNS, or Yellow Nail Syndrome, can impact multiple organs in the body, meaning complications differ based on the specific symptoms each patient has. Some people may experience stress or embarrassment due to their discolored and deteriorated nails. Further, the separation of the nail from the nail bed can cause discomfort. There are also serious, potentially fatal complications related to lung and lymphatic issues seen in YNS.
For lung-related complications:
- People often experience discomfort due to persistent, large lung fluid buildup. This can significantly decrease their daily activity levels.
- These fluid buildups are also linked with other dangerous complications like infection and breathing difficulties.
- Many people usually need multiple instances of fluid removal, which comes with risks like infection, bleeding, pneumothorax (air accumulation in the chest), and significant discomfort.
- Regular draining of these fluid buildups, which are high in protein, can cause low protein levels in the blood. This can worsen swelling and increase the recurrence of fluid buildup.
- Frequent usage of antibiotics due to recurring lung infections can lead to antibiotic resistance.
For lymphatic complications:
- Significant swelling typically impacts both lower limbs, causing difficulties in walking and weakening of the body.
- Long-term lymphedema (swelling due to lymph fluid accumulation) leads to changes in skin texture and color, which people might find unattractive. It can also lead to cellulitis (skin infection) and other infections.
- Other potential areas for fluid buildup include the space around the stomach, which may need fluid removal, the genital area causing tissue breakdown and discomfort, and around the eyes, potentially causing vision problems.
Preventing Yellow Nail Syndrome
Yellow nail syndrome is a very uncommon condition. Often, it may not be identified, or mistaken for other health issues for a long time. If a typically recommended treatment isn’t working, the whole medical team, including caregivers, should press for further tests and potential alternative diagnoses. As there’s no particular cure for Yellow Nail Syndrome, patients should understand that any treatment attempts might not work as well as hoped and could potentially have side effects.
