Is your child's skin thickening and hardening? Let's talk about Juvenile Systemic Scleroderma!

Have you noticed that your little one's skin has suddenly changed a bit? Maybe the skin has become a little rough, tight, and feels different to the touch? If you notice something like that, you should be a little concerned. Because, this can sometimes be a symptom of a condition called Juvenile Systemic Scleroderma , which we are talking about today. Don't worry, we will talk about this in detail.

What is Juvenile Systemic Scleroderma?

Simply put, Juvenile Systemic Scleroderma (also known as Systemic Sclerosis ) is a condition in which your child's skin becomes thicker and more rigid than normal. Think of it as a sheet of rubber that gradually thickens and becomes like hard plastic. It's not just limited to the skin. Sometimes, it can affect other organs inside the child's body as well. The word 'systemic' means that it can affect the child's entire body.

This is actually an autoimmune disease . That is, our body's defense system, the immune system, mistakenly starts attacking its own healthy cells. In this case of Scleroderma, when the immune system attacks skin cells in this way, inflammation or swelling of the skin occurs. Due to this swelling, the protein called collagen, which gives our skin its elasticity and strength, starts to be produced in excess. Just like a scar forms when an injury occurs, this excess collagen production causes fibrosis or scar-like hardening of the skin.

Are there main types of Systemic Scleroderma?

Yes, there are three main types. Let's see what they are.

1. Diffuse cutaneous systemic sclerosis: In this type, the child's skin thickening can be seen all over the body. Also, there may be problems with the functioning of the body's internal organs. Imagine, not only the arms, legs, and face, but also places like the chest and stomach start to thicken.

2. Limited cutaneous systemic sclerosis (CREST syndrome): This one is a bit special. It is also called CREST syndrome . CREST is an acronym formed by combining the first letters of the five main features of this condition.

• C - Calcinosis: This means that calcium deposits under the skin and forms small lumps.
• R - Raynaud's phenomenon: This is when the fingertips (hands and feet) turn white, blue, and then red when exposed to cold or when under stress. It becomes difficult to regulate body temperature.
• E - Esophageal motility dysfunction: This is when the tube that carries food from the throat to the stomach (the oesophagus) does not function properly when we swallow. This can cause things like heartburn .
• S - Sclerodactyly: Thickening and hardening of the skin on the fingers. The fingers may swell and become stiff, like sausages.
• T - Telangiectasia: Small blood vessels in the skin become enlarged, appearing like spider webs or red dots.

3. Systemic sclerosis sine scleroderma: This is when the internal organs of the body are affected without any thickening or hardening of the skin. 'Sine scleroderma' means 'without scleroderma (hardening of the skin)'. This means that although there may be no significant changes to the skin, internal organs may be affected.

Who is most likely to develop Juvenile Systemic Scleroderma? How common is it?

This condition called Juvenile Systemic Scleroderma can actually affect anyone of any age, regardless of gender. However, it is very rare for young children, that is, children under the age of 10, to develop it. The word 'juvenile' itself means that this is a condition that affects children. If you look at the statistics in a country like the United States, it is said that there are between 5,000 and 7,000 children with Scleroderma. About 2% of the total population with Scleroderma is affected by this disease before the age of 10. Another 7% face this condition between the ages of 10 and 19. So, this is considered a very rare disease .

What are the symptoms of Juvenile Systemic Scleroderma?

In this condition, symptoms can affect the child's skin and internal organs. In addition, many children with Systemic Scleroderma also experience a condition called Raynaud's phenomenon .

Symptoms affecting the skin and tissues

First, let's look at how this affects the skin and related tissues:

• The skin loses its elasticity. It becomes rough, tight, and unsightly.
• The function of the hands decreases. The skin becomes tight, especially in the fingers and palms, making it difficult to bend the hands and grasp something.
• Red blood vessels appear on the skin, especially on the hands, face, and around the nails (telangiectasias). These can look like small spider webs.
• Calcinosis is the formation of small lumps of calcium deposits under the skin or elsewhere. These can sometimes feel hard.

At first, the skin on the hands and feet may appear swollen and enlarged. Over time, the child's skin may become tight, thickened, and may develop wrinkles, sunken areas, or small pits. For example, some children's fingertips may swell like sausages, then become hard and shiny.

Symptoms affecting internal organs

Systemic Scleroderma can also affect the internal organs of a child. Symptoms may include:

• Joint swelling, stiffness, and pain. Like arthritis.
• Ulcers on the fingertips. These ulcers take a long time to heal.
• Digestive system problems. For example, heartburn, difficulty swallowing, loose stools (diarrhea), and stomach cramps.
• Respiratory problems. You may experience a persistent cough or difficulty breathing.
• Kidney problems. This can also cause high blood pressure (hypertension) .
• Feeling tired all the time. Getting tired quickly even after doing something small.
• Muscle weakness.

Most of the time, this condition is not painful. Therefore, some children may not even know they have Scleroderma. That is why a child diagnosed with Systemic Sclerosis should see a doctor regularly to check for high blood pressure, lung, kidney, gastrointestinal, and heart problems.

Raynaud's phenomenon

This is a rather unique symptom. Raynaud's phenomenon is when a child's fingertips and toes change color when exposed to cold or when they are stressed. First they turn white , then blue , and finally red . This is a symptom that can be seen in the early stages of the disease. Sometimes this can be the first clue that Systemic Sclerosis is present.

Other symptoms that may occur with Raynaud's phenomenon include:

• Feeling tired all the time
• Joint pain
• Difficulty swallowing food
• Stomach ache
• Chest inflammation
• Diarrhea
• Difficulty breathing

Why does Juvenile Systemic Scleroderma occur?

Actually, the exact cause of Juvenile Systemic Scleroderma is still unknown . However, research has shown that damage to the cells that line the inside of our blood vessels causes the connective tissue cells in the skin, called fibroblasts, to become overactive. These fibroblasts are the cells that produce proteins like collagen. So, the symptoms of Systemic Scleroderma are caused by this overproduction of collagen.

Why is a baby's skin so tight?

In Systemic Scleroderma, the skin becomes tight because of an excess of a protein called collagen in the child’s tissues. As we’ve discussed before, the immune system’s job is to protect the body from infection and disease. However, in a child with Scleroderma, the immune system overreacts and tries to protect the body with its own healthy cells – that is, it attacks its own cells . As a result, the child’s cells start to produce too much collagen. This excess collagen gets deposited in the child’s skin and internal organs, creating a hard, thickened appearance, similar to a scar.

How is Systemic Scleroderma diagnosed?

Your child's doctor will diagnose Systemic Scleroderma by considering several factors.

• Symptoms and physical signs, such as skin thickening and changes in the fingers.
• Specific changes in the skin, such as thickened areas, their size, shape, and color.
• A complete medical history and physical examination will provide information about the child's previous illnesses and whether anyone in the family has had similar conditions.

What tests are used to diagnose Systemic Scleroderma?

Various tests are done to rule out other conditions that resemble scleroderma, to determine how active the scleroderma is, and to determine whether other organs besides the skin are affected. Tests for autoimmune proteins can give some idea of ​​the course of the disease. Very rarely, doctors do a skin biopsy .

Here are some additional tests for Systemic Scleroderma:

• Blood tests: Measure autoimmune protein levels and kidney function.
• X-ray tests: Look for changes in the child's skin, bones, and internal organs (such as lungs and intestines).
• Tests to check the swallowing process in the esophagus: This means looking at the function of the tube that carries food from the mouth to the stomach.
• Pulmonary function test: A breathing test to see if the child's lungs are working properly.
• Heart ultrasound (Echocardiography): See how your baby's heart is working.

Who diagnoses Juvenile Systemic Scleroderma?

Systemic Scleroderma is diagnosed, monitored, and treated by your child's general practitioner, a rheumatologist who specializes in these conditions, a dermatologist , or a team of doctors who specialize in specific parts of the body (digestive system, heart, lungs, kidneys).

How is Juvenile Systemic Scleroderma treated?

The main goals of systemic scleroderma treatment are to stop inflammation, prevent the condition from worsening, and prevent damage to internal organs.

Protecting the skin

Protecting your skin helps to maximize blood flow to your skin and limbs. This is especially important for children with Raynaud's phenomenon . Here are some things you can do to protect your child's skin:

• Avoid injuring the affected areas, especially the fingertips and toes. Even a small cut can become a big problem.
• Protect your child's hands and feet from the cold. Keep the room warm. In the winter (in colder areas like the uplands of our country), dress your child in an extra layer of clothing, such as a hat with earflaps, mittens, and warm socks. Wool is warmer than cotton or synthetic fabrics. Also, it is better to wear several thin layers of clothing than one thick layer.
• Avoid smoking or exposing your baby to smoke.
• Avoid giving your child cold medicines that contain the ingredient pseudoephedrine.
• Protect your child from excessive sunlight. Use sunscreens as recommended by your doctor.
• Do not use astringents, body or facial scrubs, or harsh detergents on your child's skin that dry out the skin.
• Use lotions to keep your child's skin smooth as prescribed by the doctor.

Physical therapy

Basic stretching and guided exercise programs, performed by physical therapists and occupational therapists, can help maintain a child's flexibility, range of motion in the joints, muscle strength, and blood flow to the affected areas. These treatments can also help prevent joint contractures, which are stiffening of the joints. If necessary, the doctor may recommend splints or braces.

Surgery

Very rarely, orthopedic hand surgery or cosmetic surgery may be necessary to correct severe joint or skin deformities or scars. However, before surgery, the condition must be in remission , meaning it has been inactive for several years.

Your child's doctor may consider autologous bone marrow transplantation as a treatment option. Other treatments are currently being investigated.

What are the medications given for Systemic Scleroderma?

Your doctor may prescribe various medications to treat your child's condition. For example:

• Corticosteroids: Reduce inflammation in muscles, joints, and skin. Steroids can also help treat the early stages of inflammation in internal organs. However, steroids are usually not very effective in the later fibrotic stages of systemic sclerosis.
• Non-steroidal anti-inflammatory drugs (NSAIDs): For example, medications like ibuprofen and naproxen are sometimes used to reduce joint inflammation in children with arthritis. However, care should be taken not to overuse NSAIDs when kidney function is impaired.
• Other immune modulator medications: These are given to reduce the autoimmune response and prevent inflammation and subsequent scarring.
• Drugs that dilate blood vessels and improve blood flow: These are used to treat Raynaud's phenomenon .

If my child has Systemic Scleroderma, what should I expect?

There is no specific cure for scleroderma. However, you can help your child manage the condition and live with it.

Systemic Scleroderma is a chronic , long-term, and slowly progressive disease. It can last for months or years. Your child's outlook depends on how widespread the symptoms are and how much of the body (especially the skin and internal organs) is affected.

Children with lung, heart, or kidney problems are at higher risk of complications, such as joint contractures and cosmetic changes. This condition can also affect the child's bone development.

Systemic Scleroderma often does not go away completely, but it can remain at the same level for several years without getting worse.

How long can you live with Juvenile Systemic Scleroderma?

The life expectancy of a child diagnosed with Systemic Scleroderma varies depending on the severity of the condition, especially whether it has affected their internal organs. It is important to talk to your doctor about this to get a clear understanding.

Can Juvenile Systemic Scleroderma be prevented?

Since the exact cause is unknown, there is no specific way to prevent Systemic Scleroderma.

How do I care for my child who has been diagnosed with Systemic Scleroderma?

Children with Systemic Scleroderma should live as normal a life as possible. They should go to school, play, and participate in activities. In general, there are no limits to the amount of physical activity children can do (as long as it is safe). Exercise helps prevent frailty and increases muscle strength, flexibility, and endurance.

When should I take my child to the doctor?

Your child should be taken for regular checkups. Especially watch for symptoms that affect internal organs. If your child is in severe pain , has difficulty moving parts of their body, or has symptoms that are affecting their daily life, see a doctor immediately.

What questions should I ask my doctor?

Asking questions like these will help you:

• How serious is my child's condition?
• How can I help protect my child's skin at home?
• How often should my child go to physical therapy?
• What symptoms should I look out for if the disease gets worse?
• Does my child need surgery?

Are there other types of scleroderma?

In addition to the types of Systemic Scleroderma, there are two main types of Scleroderma. They are:

• Localized scleroderma: This type affects only the skin in certain areas of the body. It may also spread to the underlying muscles or bones, or it may be absent. It rarely affects internal organs.
• Systemic scleroderma (also known as systemic sclerosis): This is what we have been talking about throughout this article. In this condition, the skin can thicken anywhere on the body. In addition to skin changes, scar tissue can develop in internal organs such as the kidneys, heart, lungs, and gastrointestinal system. Children with systemic sclerosis may have limited joint movement due to widespread skin changes.

The most important things you need to remember in this article

Juvenile Systemic Scleroderma is a rare condition in children that causes the skin to thicken and harden, sometimes affecting internal organs. It is an autoimmune disease.

• Symptoms: Skin tightening, swelling of the fingers, Raynaud's phenomenon (fingers changing color in cold weather), joint pain, digestive problems, and difficulty breathing may occur.
• Cause: Although not exactly known, it is thought to be caused by a malfunctioning immune system and excessive collagen production.
• Treatment: Control the disease, protect the skin, use physical therapy and, if necessary, medication. Although there is no complete cure, symptoms can be managed.
• Most importantly: If your child has symptoms, seek medical advice immediately. It is very important to refer your child for regular medical checkups and help them live a normal life. Don't worry, you can cope with this situation with the help of doctors.

` Scleroderma, pediatric skin diseases, skin tightening, collagen, autoimmune diseases, Raynaud's phenomenon, CREST syndrome