Most of us know collagen is an important protein in the body. It is responsible for keeping your skin tight, supple, and young-looking. As you grow older, this collagen production decreases, leading to wrinkles and many other age-related skin conditions. What many don’t know is collagen is also responsible for keeping your joints young. This is why the older you get, the more you are prone to joint pains and other related issues. An imbalance in collagen production can lead to various conditions. Scleroderma in children is one condition related to abnormal collagen production.
Normally, collagen production is at its peak during childhood. But sometimes excess collagen production can happen which can be very harmful, leading to a chronic illness called scleroderma. Has your child been diagnosed with Scleroderma? Not sure what to do? Here is everything you need to know about Scleroderma in children.
In This Article
- What is Scleroderma?
- What is Pediatric Scleroderma?
- Different Types of Scleroderma in Children
- Causes of Scleroderma in Children
- Signs and Symptoms of Pediatric Scleroderma
- Diagnosis of Scleroderma in Children
- Treatment of Pediatric Scleroderma
- Complications of Scleroderma in Children
- When Should You Consider Seeing a Doctor?
- What Can You do as a Parent?
What is Scleroderma?
Scleroderma is an autoimmune disease that causes excess production of collagen. When there is too much collagen, it can cause you more harm than good and can affect the organs. Scleroderma can cause inflammation in the skin and affect the internal organs due to excess collagen. Sadly, this condition is not limited to adults and affects children too
What is Pediatric Scleroderma?
The onset of scleroderma in children and teens is known as pediatric scleroderma. Pediatric Scleroderma is an autoimmune condition and chronic illness with no permanent cure but can be kept under control with effective treatment. The condition starts as a thickening of the skin which can also look like swelling. Over time, this swollen part of the skin will get hard and shiny. It will feel waxy and tight.
For some children, the skin undergoes this change in patches, leaving them with bits and patches of hard, shiny skin in various parts of the body. For others, the skin on the entire body can change and become waxy and hard. A parent with scleroderma has a higher possibility of passing the defective gene to the child. While Systemic Scleroderma is more common among adults, localized scleroderma is more common among children.
Different Types of Scleroderma in Children
Pediatric scleroderma can be classified into two types
1. Localized Scleroderma
Also known as Morphea, this condition causes patches of skin to become hard on the trunk, head, arms, and legs. Children get affected by this type more frequently than with Systemic scleroderma. LS (Localized Scleroderma) is hardly seen among adults. Among children, LS causes extensive involvement of the skin, unlike Systemic Scleroderma. The disease can be active for a longer period in children than in adults. For an adult diagnosed with LS, the disease can be active for about 3 to 5 years. For a child, it can be active for a period of 7 to 10 years.
Localized Scleroderma can be further classified into
a) Circumscribed Morphea
This is the type of morphea that results in discoloured patches of skin all over the body. In some children, it can be just one patch and in some, it can be a few patches. These patches can be of different sizes. They will typically be pale yellow in the center and red around the borders. During the active period, these patches can spread and become larger. When inactive, they can flatten out and treatment can make them asymptomatic. Sometimes these patches can even be deep and reach the subcutaneous tissues.
b) Generalized Morphea
In this type of scleroderma, more than four morphea plaques can be seen around the trunk, head, neck, and arms of the child. These thick patches of plaques can blend into one another leading to a more severe impact. Pansclerotic morphea affects most of the body and is a severe form of generalized morphea.
c) Linear Scleroderma
This variant is more common among young children below the age of 10. It causes thick, tight bands of skin around the trunk, face as well as buttocks. These usually appear as just a single band of thick skin. However, mixed patches of morphea can also be seen along with this band of tight skin.
This type can also affect the muscles and the bones of the child. If the linear scleroderma affects the limbs, the child might suffer from underdeveloped limbs, which can affect their growth and lead to a disability.
2. Systemic Scleroderma
This type of scleroderma is very rare among children and seems to occur more frequently among adults. It affects the joints, internal organs, and the skin. This condition is an autoimmune disease that can also cause blood vessel abnormalities. Systemic Scleroderma is the variant that causes more complications in the patient. This is why a child diagnosed with SS (Systemic Scleroderma) must be under constant observation. They need to visit the doctor regularly to ensure the disease does not progress enough to cause any damage inside the body.
Systemic Scleroderma can be further classified into three types:
a) Limited Scleroderma
As the name suggests, skin hardening is limited to the fingers and in some cases can reach the arms and face. It usually does not cause any damage to the internal organs and the patient can lead an almost normal life.
b) Diffuse Scleroderma
Excess collagen production will cause the skin to thicken in large areas of the body. Over time this type can damage the organs, including the gastrointestinal tract, lungs, kidney, and heart.
c) Sine Sclerosis
This type has all the features of systemic scleroderma that affect the organs but not the skin.
Causes of Scleroderma in Children
Pediatric Scleroderma is an autoimmune disease, meaning it is caused due to an issue with the immune system. A disease is classified as autoimmune when the immune system attacks the body instead of protecting it. It is the immune system’s role to protect the body against harmful bacteria, viruses, and other infections by producing relevant antibodies. When the immune system malfunctions, it produces antibodies to attack the body. There is no proven cause for this disease. Doctors and scientists believe it could be genetic or environmental factors. A child, whose have scleroderma, has a higher chance of being affected by pediatric scleroderma.
Research is still underway to understand more of this disease, its variants, and how it affects children and adults differently.
Signs and Symptoms of Pediatric Scleroderma
A little extra skin thickening is not bound to cause any concern among parents. A little joint pain in a child does not raise any eyebrows either unless the pain does not subside. It is easy to miss scleroderma and mistake it for other skin-related issues or joint injuries.
Common Symptoms of Pediatric Scleroderma
- Joint pains.
- Swelling or thickening of fingertips.
- Sores on knuckles over the fingertips.
- Calcium bumps on various parts of the body.
- Spider veins.
- Raynaud’s phenomenon – fingertips can get pale or blue and tingly when emotionally upset or exposed to the cold for a long time.
- Scarring of the skin can lead to frozen fingertips, elbows, or even wrists.
- Dark, shiny, and taught skin, on large areas of the body. They can even cause issues with movement as they affect the joints.
- Shortness of breath due to scarring of the lung.
- Heart problems like arrhythmia or heart failure.
- Grating noise caused by the movement of inflamed tissues.
- Kidney disease
Additional Symptoms of Pediatric Scleroderma
As localized Scleroderma is more common among children, you need to watch out for these additional symptoms:
- Skin colour changes – it can get lighter or darker
- Shiny and thick patches of skin
- Tightness in joints
Remember, these are just possible symptoms. It is not necessary for a child with scleroderma must experience many or all the symptoms mentioned. Similarly, just because a child shows a few of these symptoms, you cannot diagnose it as scleroderma. Always consult a qualified doctor and go for a second opinion if need be. Do not conclude on any diagnosis unless it is done by an experienced medical professional – a Pediatric Rheumatologist would be the right doctor in this situation.
Diagnosis of Scleroderma in Children
The symptoms mentioned for Localized Scleroderma, which is more common among children, are very common symptoms among children. A child having a patch of different colored skin or some joint stiffness may not cause any real worry. So, it is easy to miss these symptoms or mistake them for some other illness.
When the doctor suspects scleroderma, they will check
1. Blood and Urine Sample
The blood and urine samples are collected to check for issues with blood count, liver or kidney function.
2. ECG
ECG is done to check for any damage to the heart caused by scleroderma. The ECG helps record the electrical activity of the heart. Any gaps, difference in the pace of heartbeats are sure clues of scleroderma affecting the heart
3. Echo
It is done to check the valves and movement of the heart, for any abnormalities.
4. X-Ray of the Chest
X-Ray are taken to detect any changes in the lungs caused by scleroderma.
After the diagnosis of scleroderma, the doctor will ask for an antibody test. This test will help identify the type of Scleroderma the child is affected with. It is very important to find this at the earliest so that the appropriate treatments can be started without further delay.
Treatment of Pediatric Scleroderma
When diagnosed early, effective treatment can help keep the condition under control and prevent severe damage. It can also help the child lead a close to normal life. If not caught in time or if the disease is severe, it can lead to many complications.
After diagnosis, the doctor will decide on the treatment based on the following factors:
- Symptoms
- Age
- Severity
- Health of the child
- Family health history
Some of the treatment options are:
1. Helping Reduce Pain
Pediatric scleroderma can cause a lot of pain and discomfort for the child. As the joints thicken and reduce fluidity and movement, it can be hard for a child to do even their regular activities. In such a case, the doctor might give medicines to ease the pain. Medicines like corticosteroids or non-steroidal anti-inflammatory medicines can also be given in severe cases.
[Read : Steroids in Children – Are They Always Harmful?]
2. Slowing Down Growth
The growth of the extra tissues can be slow or fast in some. If caught early, medicines can reduce the growth pace. The doctor might give medicines to arrest or slow the skin thickening process. This can help delay the damage caused to internal organs.
3. Treating Symptoms
The symptoms can be different for different children. If any of the symptoms are strong or causing other issues, the doctor will address these symptoms first. They will prescribe medicines to control these symptoms first, to avoid any other health complications or damages to the body.
4. Weaken the Immune System
Pediatric scleroderma is an autoimmune disease, meaning the immune system is hyperactive and attacks the body. The doctor might prescribe Immunosuppressants to control the immune system. This can help reduce the immune system’s attack on the body but can also make the child vulnerable to other infections, as the immune system will be weak.
5. Physical Therapy
As joints are affected, physical therapy can help improve mobility and reduce pain. The therapists will give exercises to strengthen the affected joints, to help the child be more mobile, active, and lead a better quality of life.
Complications of Scleroderma in Children
The impact and severity of the disease differ from child to child. Though treatment can help alleviate the pain and reduce the symptoms, it cannot cure the disease completely.
Some of the possible complications of pediatric scleroderma are:
- Arthritis
- Seizures
- Thickened skin that feels tight
- Change in vision
- Decreased lung function
- Damages to the kidney and the heart
- Heartburn or gastrointestinal issues
Please understand that these are only possible complications. Not all children suffering from pediatric scleroderma need to experience these symptoms or have complications at a later stage. When the disease is caught early and is treated well, it can be kept under control for a longer period.
Localized Scleroderma can be active for 7 to 10 years in a child. The longer the disease is active, the more damage it can cause to the body. The excess collagen production can result in deposits on the organs and the joints, thereby damaging the organs and making it more difficult to move.
[Read : Juvenile Idiopathic Arthritis in Children]
When Should You Consider Seeing a Doctor?
If your child shows any of the symptoms mentioned earlier, you need to consult a doctor immediately. It is very important to get an early diagnosis and start treatment, to reduce damage to the organs and other parts of the body.
If your child is already diagnosed with pediatric scleroderma, here are some instances that warrant a doctor’s consultation immediately:
- New symptoms crop up.
- You see new patches of skin on your child’s body,
- Existing symptoms get severe or worse, causing pain and discomfort for the child.
What Can You do as a Parent?
Being a parent to a child suffering from pediatric scleroderma is not an easy task. It can be physically and mentally very exhausting. If your child is experiencing restrictions in movement, it can affect their quality of life in many ways. They may not be able to enjoy a “childhood” like other children.
7 Ways to Help You Child Deal With Scleroderma
- Consult a good doctor and get recommendations for physical therapists
- Start physical therapy early and follow through even after pain and symptoms subside
- Work with the therapist and ensure you help your child do the exercises and follow instructions even at home, to help ease their pain and improve physical movements
- Plan activities your child can handle. You can organize suitable games with other children so that your child does not feel left out.
- Find a support group with other children who suffer from pediatric scleroderma. They won’t feel alone and they will accept reality better. This group can also be their safe space to share their difficulties and insecurities.
- Talk to the school, explain the situation to the teachers, and ensure they understand your child’s limitations.
- Talk to your child; listen to their insecurities and troubles. Though you may know it all and might have heard it a number of times, do be patient and let your child get it all out. It is a very difficult phase for them. When they are not able to live like other children, it can make them feel lonely and affect their mental health to a great extent.
Pediatric Scleroderma is an autoimmune disease with no permanent cure or apparent cause. It can be difficult to both diagnose and treat this condition. It can take a toll on both the physical and mental health of the child. With timely diagnosis, consistent treatment, and medications, the child can lead a good life without any major compromises.
Read Also: Systemic Lupus Erythematosus(SLE) in Children by Dr. Sagar Bhattad