Lymphedema Tarda: Causes, Symptoms & Treatment

Several medical conditions and disorders can cause lymphedema, and symptoms can start at any age. Different types of lymphedema may present different symptoms, so getting a proper diagnosis is a vital part of managing lymphedema. Your doctor can distinguish between lymphedema tarda, lymphedema praecox, and other types of lymphedema. If you recently developed lymphedema in your adult life, here’s what you need to know about lymphedema tarda.

What Is Lymphedema Tarda?
What Is the Difference Between Lymphedema Praecox and Lymphedema Tarda?
What Causes Lymphedema Tarda?
What Are the Symptoms of Lymphedema Tarda?
How Is Lymphedema Tarda Diagnosed?
How Do You Treat Lymphedema Tarda?
Wrapping Up: Lymphedema Tarda

What Is Lymphedema Tarda?

Lymphedema tarda is a type of hereditary lymphedema that’s characterized by the age of onset. Hereditary lymphedema — a type of primary lymphedema — can be present at birth, during childhood, or during adulthood. Lymphedema tarda is a form of primary lymphedema that develops after the age of 35.¹

Unlike secondary lymphedema, lymphedema tarda is caused by a genetic lymphatic disorder. Getting a diagnosis is important because it helps your doctor or specialist understand how to treat your lymphedema appropriately to better manage your symptoms and decrease the risk of progression.

What Is the Difference Between Lymphedema Praecox and Lymphedema Tarda?

Primary lymphedema is due to a genetic mutation that causes malformation of the lymphatics in the embryo. There are different types of lymphedema that develop at different ages.

Type I lymphedema is also known as congenital lymphedema, and it occurs between birth and two years old. Even though the lymphatic dysfunction that causes hereditary lymphedema may be present at birth, swelling doesn’t always start at an early age. In rare cases, lymphedema swelling may be present at birth or shortly after.¹

Type II lymphedema, or lymphedema praecox, occurs between 2 and 35 years old. While the lymphatic system issues are present at birth, signs of lymphedema do not present themselves until a later age. Swelling caused by lymphedema praecox generally starts to present around the age of puberty, although that can vary. The severity of lymphedema praecox symptoms can also vary.¹

Lymphedema tarda is also known as type III lymphedema, which describes hereditary lymphedema that occurs after the age of 35. While research is minimal, it suggests that lymphedema tarda is caused by a genetic mutation in the FOXC2 gene.¹

What Causes Lymphedema Tarda?

Determining the exact cause of lymphedema tarda can be difficult, but research suggests it’s a hereditary condition that is passed on through genetics.

Although there are no exact known lymphedema tarda causes, the condition has been associated with a mutation of the FOXC2 gene.² This gene is responsible for the development of the valves in your veins and lymphatic system.

What Are the Symptoms of Lymphedema Tarda?

Recognizing symptoms is a crucial step in getting proper lymphedema tarda treatment. In this section, we’ll talk about some of the common lymphedema tarda symptoms and how they typically present. Keep in mind that you may not experience every symptom on the list — it depends on the severity of your condition.

• Swelling: Swelling is the most common symptom associated with lymphedema tarda. This swelling is caused by an impaired lymphatic system that is unable to process built-up fluid in the tissues, and it can range from minor to severe. Managing swelling is the primary focus of lymphedema treatment. Without regular treatment, swelling may progress and lead to complications, including ambulatory dysfunction and changes to your skin.
• Pain: Pain is another common lymphedema tarda symptom that can vary from patient to patient. Some people experience minimal pain with lymphedema, while others may only experience swelling and discomfort. The level of pain can fluctuate based on the severity of your swelling, which is why it’s important to consult with your doctor or lymphedema therapist about the steps you can take to minimize swelling and prevent complications.
• Numbness: In some cases, lymphedema may cause numbness and tingling in your extremities. This numbness starts in the area where swelling is present, but it may affect the surrounding area as swelling progresses.
• Pitting edema: If you press a finger on the swollen part of your body and it leaves an indentation, you have pitting edema. When pressure is applied to healthy skin, it rebounds — or returns to its normal shape — when you stop pressing on it. Pitting edema is graded based on the depth of the indentation and how long it takes for the skin to rebound. Pitting is a common symptom with any type of edema, but you should see a doctor or lymphedema specialist if your pitting continues to progress.
• Changes to the skin: In addition to swelling, you may notice visible changes to your skin as a result of lymphedema tarda. Your skin may harden or become thicker, known as fibrosis, and in severe cases, you may notice wart-like patches and edema blisters on your skin where swelling occurs.
• Decreased range of motion: The early stages of swelling can cause slight pain and discomfort, but severe swelling can eventually lead to a decreased range of motion. People with lower body lymphedema may have a difficult time walking and performing other physical activities, while people with upper body lymphedema might have trouble with certain arm movements. An occupational or physical therapist can help you restore your range of motion through guided exercises, with swelling reduction playing a key role in getting your range of motion back.
• Discomfort: Discomfort is a common symptom associated with lymphedema, and it can vary substantially depending on how severe your swelling is. Discomfort caused by lymphedema typically starts off mild — you may notice some skin tightness or a heavy feeling. As swelling progresses, lymphedema can make physical activities difficult and cause discomfort when you’re sitting or lying down.
• Increased risk of infection: People with lymphedema are more prone to infections, and those infections, like cellulitis, can lead to medical emergencies if they’re not treated properly. If you have lymphedema, you should talk to your doctor or specialist about skin care tips. Keeping your nails cut short, moisturizing, and wearing sunscreen can help you avoid skin damage that can lead to infections.

How Is Lymphedema Tarda Diagnosed?

If you’re experiencing lymphedema tarda symptoms, getting a diagnosis is a crucial first step in managing lymphedema. Your doctor can help you rule out other potential medical conditions and create a lymphedema tarda treatment plan based on your diagnosis.

Diagnosing lymphedema tarda starts with a thorough exam to rule out the potential for underlying health conditions. Your symptoms could be caused by conditions like deep vein thrombosis (DVT) or congestive heart failure. Chronic venous insufficiency (CVI) can also lead to fluid imbalances, which over time, may cause lymphedema. Your doctor will also rule out secondary lymphedema, which can be caused by cancer surgery or cancer treatments, chronic venous disease, injuries, or infections.

Once your doctor has ruled out other medical conditions, they’ll use a variety of tests and techniques to diagnose you. A lymphoscintigraphy can be used to see how fluid flows throughout your lymphatic system, and MRI and ultrasounds can help your doctor pinpoint lymphatic system blockages.

Your healthcare provider may refer you to a certified lymphedema therapist for specialized treatment. Using your medical history, tests, and the age of onset, a specialist can diagnose, classify, and treat your lymphedema.

How Do You Treat Lymphedema Tarda?

Because lymphedema is a chronic condition, lymphedema tarda treatment is focused on reducing and managing swelling to prevent complications and keep your lymphedema from progressing. Basic treatment methods, including compression, can usually control swelling. In rare cases of severe lymphedema, surgical intervention may be required. Here’s what you can expect when it comes to lymphedema tarda treatment:

• Complete decongestive therapy (CDT): Certified Lymphedema Therapists (CLT) are trained in complete decongestive therapy, which is a comprehensive approach to managing swelling and other symptoms. CDT combines compression, skin care, exercise, and manual lymphatic drainage to help reduce swelling. When you combine CDT with a healthy diet and other treatment methods, you can reduce risks, such as cellulitis and ulcers.
• Manual lymphatic drainage: Manual lymphatic drainage (MLD) is a massage technique that’s used to encourage lymphatic drainage. Your lymphedema therapist or occupational therapist can perform a lymphatic drainage massage for you, or you can learn to perform your own massage at home. Manual lymphatic drainage is easy, safe, and time-tested. The process typically takes about 20 minutes and plays a key role in reducing and managing swelling.
• Exercise: Exercise offers several benefits for lymphedema patients. Regular exercise helps you manage your weight, as excessive weight can cause lymphedema symptoms to worsen. Exercising also helps encourage the flow of fluid in your body, which can help improve your circulation and lymphatic function. Your therapist can show you special exercises for lymphedema that provide relief without causing pain or discomfort. If you work out or enjoy playing sports, talk to your doctor or specialist to make sure those exercises are safe with lymphedema.
• Skin care: A good skin care routine helps you avoid several lymphedema complications — especially infection. After you get out of the bath or shower, you should thoroughly dry your skin and apply a moisturizer daily. Keeping your nails trimmed helps prevent accidental scratches that can turn into infections. If you’re going out in the sun, make sure you wear SPF 30 or higher sunscreen to protect yourself from sunburn. Avoiding injuries is the easiest way to avoid infections with lymphedema.
• Compression garments: Compression is an essential part of complete decongestive therapy, and compression garments are a popular choice. Your doctor or specialist may prescribe compression socks or sleeves to gently guide fluid away from the swollen parts of your body. Compression garments are available in different compression support levels, so it’s important to talk to a doctor or lymphedema therapist before wearing them.
• Pneumatic compression devices: If you’re looking for targeted relief and compression you don’t have to wear all day, pneumatic compression devices are an excellent choice. Devices like the Flexitouch Plus System and Entre Plus System from Tactile Medical use pneumatic compression to target your swelling and guide fluid away from the area. You can choose between different devices based on the location of your swelling, with lower body, upper body, and head and neck devices available.
• Nutrition: Proper nutrition is an essential part of your overall health. Eating right and exercising can help you maintain a healthy weight. As part of your lymphedema tarda diet, your specialist may recommend lowering your salt intake, cutting out caffeine and alcohol, and eating plenty of healthy protein.
• Elevation: Elevating your swollen arm or leg allows gravity to guide the fluid in your lymphatic system away from the blockage. You can adjust your bed to slightly elevate your legs or use a support device to keep your arms or legs elevated.

Wrapping Up: Lymphedema Tarda

Lymphedema tarda can be an unexpected development in people, as it typically occurs after the age of 35. Getting a proper diagnosis and taking steps to manage your swelling can help you keep lymphedema from progressing and lower your risk of complications. With regular treatment and management, you can live a healthier life.
Compression is an essential part of reducing and managing swelling, and Tactile Medical can help. The Flexitouch Plus System and Entre Plus System are designed to provide targeted relief for swelling caused by lymphedema, helping you manage your symptoms. Ask your lymphedema specialist or contact Tactile Medical to learn more about our solutions.

References

1. Nitti, Matthew, et al. Definition, Incidence and Pathophysiology of Lymphedema. Principles and Practice of Lymphedema Surgery. 2016. Accessed September 7, 2023. https://www.sciencedirect.com/topics/medicine-and-dentistry/lymphedema-tarda
2. An approach to familial lymphedema. National Library of Medicine. Accessed September 7, 2023
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6297692/