Lipodermatosclerosis

What is Lipodermatosclerosis?

Lipodermatosclerosis, sometimes called sclerosing panniculitis or hypodermitis sclerodermaformis, is a long-term skin inflammation issue. This condition causes the skin and the tissue just underneath the skin (subcutaneous tissue) on the lower parts of your legs to harden and thicken, similar to how scar tissue forms. This was first described by Huriez and his team in 1955, but the exact cause and process of this problem still aren’t completely clear.

Several theories have been suggested on how this problem happens. Some evidence points towards a link between lipodermatosclerosis and issues with the veins in the legs not working properly (venous insufficiency), which creates higher pressure in these veins. This results in different substances, including a protein called fibrinogen and other molecules that cause inflammation, leaking from the capillaries (tiny blood vessels) into the surrounding skin and tissue. There may also be issues with the process that breaks down fibrin (a clotting protein), known as fibrinolysis.

Over time, the inflammation causes the tissue under the skin to harden (fibrosing panniculitis), leading to lipodermatosclerosis’s classic look – a lower leg that has the shape of an upside-down champagne bottle. This is how a leg looks when lipodermatosclerosis has become quite advanced; the condition can show itself in different ways depending on how far it’s developed. Typically, doctors diagnose this based on its key features. If the diagnosis isn’t clear, a small sample of the affected tissue can be taken for microscopic examination (histological examination) to confirm if it is lipodermatosclerosis.

What Causes Lipodermatosclerosis?

Lipodermatosclerosis usually occurs due to an issue known as chronic venous insufficiency, which can happen when the veins’ valves don’t work properly, there’s a blockage in the veins, or the calf muscles aren’t functioning well. Some of the main risk factors for developing this type of vein problem, and thus lipodermatosclerosis, are obesity, aging, having had a deep vein blood clot, a family history of vein problems, and smoking. People who don’t exercise much or who spend a lot of time standing at work are also at a higher risk. In the same way, if the ankle is immobile for a long time, it can cause symptoms similar to chronic venous insufficiency.

A recent study used certain types of scans and tests to show that most patients with sudden onset lipodermatosclerosis had underlying vein problems, even if they didn’t show typical signs of chronic swelling due to poor blood flow. This supports the idea that lipodermatosclerosis comes from chronic vein disease. Though this vein problem seems to be the main reason for developing lipodermatosclerosis, other factors might contribute too. For example, there could be a link between abnormalities in blood clot and dissolving mechanisms, such as deficiencies in proteins C and S, and the development of lipodermatosclerosis. However, this possible relationship isn’t fully understood yet.

Risk Factors and Frequency for Lipodermatosclerosis

Lipodermatosclerosis, a certain skin condition, often affects women, particularly those with limited mobility and a high body mass index. This condition tends to show up in people who are middle-aged or older. Generally, it is women over 40 years of age who are diagnosed with this condition, but it can start as late as age 75.

Signs and Symptoms of Lipodermatosclerosis

Lipodermatosclerosis is a condition that generally affects people with a history of long term issues with vein function. The affected person might also have certain signs such as vein enlargements, scars from previous ulcers, and swelling of the lower limbs. Darkening of the skin is another common sign due to iron depositing in the skin.

Usually, this condition affects both limbs, however, in some cases, it could only affect one limb. The duration of the disease can be classified into acute, which is less than a month, subacute which is between 1 month to 1 year, and chronic which is over a year. Subacute lipodermatosclerosis, however, can show the features of both acute and chronic forms.

The acute phase appears as red patches on the skin which may be misunderstood as a bacterial infection resulting in initial treatments being antibiotics. Generally, these symptoms are larger and more noticeable in the inner lower leg.

The chronic phase is often associated with clear, thick, dark patches on the skin which are not tender to touch. These patches are firm and the overall appearance of the leg can resemble an inverted champagne bottle due to the swelling in the lower and upper parts of the leg and the narrowing at the ankle region due to buildup of fibrous tissue.

Testing for Lipodermatosclerosis

If your doctor suspects you have a skin condition called lipodermatosclerosis, they will first take a detailed account of your medical history and perform a thorough physical examination. Generally, the diagnosis of lipodermatosclerosis is made based on these clinical notes, and further tests are usually not necessary.

However, if the standard treatment isn’t working or your symptoms aren’t following the usual pattern, your doctor might consider doing a biopsy and looking at your tissue under a microscope for further investigation. Normally, doctors try to avoid biopsies on the lower legs because wounds tend to heal slowly in this area.

A biopsy for lipodermatosclerosis can be a little tricky. This is because the disease mainly affects the layers of skin below the surface, where it causes fibrous thickening. Tiny biopsy samples might not reach this deep, so it’s necessary to get a larger sample which could be obtained through a type of biopsy called an elliptical excisional biopsy or a bigger punch biopsy. However, because patients with this condition often have slow wound healing, a type of biopsy that creates a less noticeable scar, such as an excisional biopsy, is usually preferred. Also, to maximize chances of capturing relevant tissue samples, the biopsy should be taken from the edge of the affected area, not the center.

Otherwise, laboratory, radiographic, or other diagnostic tests are not typically needed for patients suspected to have lipodermatosclerosis. That said, if the biopsy results are unclear, or if you have symptoms that might suggest a more serious condition, additional testing may be required.

Treatment Options for Lipodermatosclerosis

Most of the information about treating lipodermatosclerosis, a skin condition affecting your legs, comes from individual case studies as there haven’t been many large clinical trials. However, the generally accepted initial treatment methods are compression therapy, which involves wearing tight stockings, and keeping your legs elevated. These approaches can help minimize swelling and reduce pain by improving blood flow in your legs.

For an acute or fast on-set of lipodermatosclerosis, creams containing corticosteroids can help reduce symptoms. If the disease is limited to a few hard, thickened spots, this medication might be injected directly into the plaques. If the condition affects a larger area of skin, it’s usually better to use the cream. Adding regular physical activity and losing weight, if you’re overweight, can also help manage lipodermatosclerosis. Physical activity enhances the flow of blood from your lower legs by strengthening calf muscles, and weight loss is particularly helpful if you’re obese.

For chronic or long-lasting lipodermatosclerosis, or acute disease that doesn’t respond to initial treatments, oral anabolic steroids like danazol and oxandrolone may be used. These medications can help to reduce your pain, decrease skin hardness and limit how far the disease spreads by helping your body break down blood clots that might be contributing to the poor blood flow in your legs. However, they can have adverse effects like sodium retention, modifications to your fat levels in the blood, liver toxicity, and masculinizing effects in females. For this reason, doctors may advise against their use for patients with severe high blood pressure or heart failure.

When systemic treatments are not enough, other interventions may be used. Ultrasound therapy, a procedure first described over 25 years ago, has shown promising results in reducing skin hardness and redness in recent trials. When paired with compression stockings, ultrasound therapy has the potential to reverse the disease’s fibrotic or ‘hardening’ effects and inflammatory changes. It might be recommended if the initial treatments aren’t sufficient. Other treatments like capsaicin cream can help manage chronic pain, but they don’t prevent the disease from getting worse. If you have lipodermatosclerosis and don’t respond well to other treatments, you might require a visit to a vascular surgeon or interventional radiologist for treatment of underlying varicose veins.

What else can Lipodermatosclerosis be?

When doctors are figuring out if a patient has a skin condition called lipodermatosclerosis, they might need to rule out many other conditions, depending on the stage of the disease. In the beginning stage, lipodermatosclerosis can easily be mistaken for conditions like cellulitis, erythema nodosum, fat necrosis caused by an injury, or other types of panniculitis, which all can cause skin inflammation.

In cases where the lipodermatosclerosis isn’t responding to first-line therapies or the patient’s condition doesn’t follow the expected pattern, a biopsy or tissue sample from the lesion can be taken to further narrow down the possible diagnoses.

• Cellulitis typically shows inflammation around blood vessels, mainly made up of a type of white blood cells called neutrophils.
• Erythema nodosum shows inflammation of the fatty layer of the skin and a mixture of infiltrating cells without blood vessel inflammation, and is known for a blob-like infiltrating cells with giant cells.
• Fat necrosis, or the breaking down of fat tissue, usually presents as separate lesions, often related to a history of physical trauma.

In the later stages of lipodermatosclerosis, doctors may consider other conditions that they wouldn’t typically think about in the beginning stage. This includes conditions like morphea, necrobiosis lipoidica, and nephrogenic systemic fibrosis.

• Morphea usually shows up as hard plaques on the skin that are ivory in color with a characteristic purple-colored ring. Under the microscope, it shows thick collagen bundles without lipomembranous dystrophy.
• Necrobiosis lipoidica presents itself as symptom-less patches or yellow or red plaques, and under the microscope, it shows an alternating layered inflammatory process.
• Nephrogenic systemic fibrosis is associated with exposure to a contrast agent called gadolinium in patients with impaired kidney function.

What to expect with Lipodermatosclerosis

Studies have shown varying results when it comes to the timeline and outcome of a condition called lipodermatosclerosis. This is a skin condition usually found on the legs and often linked to problems with vein function.

A recent study which involved almost 30 patients showed that all patients’ acute-phase symptoms were completely healed by using compression bandages. However, after stopping the use of the compression therapy, about half of the patients progressed to the chronic stage of lipodermatosclerosis over the ensuing year. The remaining half did not show any further advancement of the disease.

Like many other conditions related to vein insufficiency, lipodermatosclerosis is often chronic and tends to come back repeatedly. Even though treatment can help ease symptoms and slow down disease progression, lipodermatosclerosis usually takes a long time to heal and often keeps progressing.

Possible Complications When Diagnosed with Lipodermatosclerosis

The long-term phase of lipodermatosclerosis, a skin condition that affects your legs, often seen as the final stage before ulcers begin to form. This stage is marked by poor wound healing due to continuous inflammation and scarring. However, it’s not unusual for venous ulcers (sores that are slow to heal) to occur alongside lipodermatosclerosis. Many studies have shown a direct link between the degree of skin hardening and the risk of developing ulcers and healing potential. Even a minor scrape or itch can lead to ulcers in people with significant skin hardening.

Another possible issue linked to lipodermatosclerosis is pain in the lower parts of your body. A recent study found that pain is the most common ongoing symptom observed in people with lipodermatosclerosis. The early stage of lipodermatosclerosis usually comes with severe, burning pain. As the condition advances, people may develop a constant, aching pain in their lower legs.

Common side effects of lipodermatosclerosis:

• Difficulty in wound healing due to continuous inflammation and scarring
• Risk of developing venous ulcers
• Skin hardening leading to ulcers, even from a minor scrape or scratch
• Developing lower body pain
• Constant, aching pain in lower legs in advanced conditions

Preventing Lipodermatosclerosis

Even though we don’t fully grasp how it happens, we know that there’s a solid connection between a condition called chronic venous insufficiency and the development of a skin issue known as lipodermatosclerosis. Regular check-ups for patients with chronic venous insufficiency are vital. It helps in spotting and managing potential issues early on, which can improve how you fare in the long run.

Helpful lifestyle changes include being active and keeping your legs raised to limit swelling among people suffering from chronic venous insufficiency. If these actions don’t quite do the trick, compression socks can be added. These socks should be worn correctly, so your doctor will teach you how to use them – bear in mind that consistently wearing them has been shown to reduce swelling, aid in healing lower leg wounds, keep blood flowing adequately, and decrease the chances of issues coming back.

Interestingly, conditions like chronic venous insufficiency and lipodermatosclerosis are more likely to occur in individuals who smoke or carry excess weight. Therefore, weight loss and quitting smoking are also encouraged as a part of treatment.

If the initial treatments don’t alleviate swelling or skin changes, an extensive duplex exam of the lower legs should be done. This exam confirms a diagnosis of chronic venous insufficiency by determining if blood is flowing backward in the veins. Only specially trained professionals like vascular sonographers, interventional radiologists, or vascular surgeons conduct this exam. For best results, the patient should stand up during the exam to increase vein visibility because this position creates pressure inside the veins similar to when they are standing.

If you have varicose veins, exhibit signs of lipodermatosclerosis, or have a history of skin ulcers, you should have them addressed promptly. Doing so can help stop the disease from getting worse and reduce further skin changes.

Any sudden skin changes should be reviewed by a dermatologist or another health professional. Lipodermatosclerosis can cause painful skin changes that look like cellulitis, a bacterial skin infection. If untreated, this condition can cause tightness in the skin and result in recurring ulcers and permanent scars.

Patients diagnosed with lipodermatosclerosis or chronic venous insufficiency must look after their skin’s health by routinely checking their skin for signs of injury and infection. Regularly applying moisturizers can help prevent painful cracks or ulcers on the skin. If ulcers develop, consider seeing a wound care specialist for proper care.

Finally, it’s important to understand that lipodermatosclerosis and venous disease are long-term conditions. Sticking to the prescribed treatments and regularly checking in with your healthcare providers is necessary for preventing these conditions from worsening and causing complications.