Is your skin thickening and becoming hard? Let's learn about Scleroderma!

Do you sometimes feel like your skin is a little thicker and tighter than usual? Or do you have joint pain or morning stiffness? These are things we might not pay much attention to. However, they could also be symptoms of a rare condition called Scleroderma . Let's talk about this in a little more detail today, shall we?

What is Scleroderma?

Simply put, Scleroderma is a rare condition in which the tissues in your body become thicker and harder than normal. Most often, it affects the skin. But sometimes, the condition can affect any other tissue in the body.

This is actually an autoimmune disorder . Now you might be wondering what an 'autoimmune disorder' is. Our body has an army-like system that protects us from disease, called the immune system. But in this situation, that system goes wrong, and instead of fighting off foreign germs, it starts attacking our own good cells. It's as if we can't tell who's our own and who's the enemy. Even doctors still can't figure out exactly why this happens.

If you have scleroderma, your immune system signals your body's cells to make too much of a protein called collagen . Our bodies actually need collagen. It's what makes the strong, healthy connective tissue that supports our organs. But when it builds up too much, the skin and other tissues become thicker and more fibrous than normal.

Scleroderma is a chronic condition . This means that you may have to live with these symptoms for a long time, perhaps for the rest of your life. Also, if it affects the tissues of your internal organs, it can cause life-threatening complications. If you feel like you are having a heart attack, have difficulty breathing, or have difficulty swallowing, call 911 (or your local emergency number) immediately, or go to a hospital emergency room.

If you have pain or stiffness in your joints, especially around your fingers and toes, you should definitely see a doctor.

What are the main types of Scleroderma?

Doctors divide scleroderma into two main types:

1. Localized Scleroderma: 'Localized' means confined to one area. This type affects only one part of the body (usually the skin). It causes patches or streaks of thickened, waxy skin. The best part is that localized scleroderma can sometimes resolve on its own . It also doesn't usually spread to other parts of the body.

2. Systemic Sclerosis: This type can affect other organs in addition to the skin. It can affect your respiratory system (the organ that helps you breathe and smell) and your digestive system (the organ that turns the food we eat into energy). If scleroderma affects your ability to breathe or absorb nutrients, you are more likely to develop serious complications. It can even be fatal. Systemic Sclerosis also has three subtypes – diffuse, limited, and sine sclerosis.

Diffuse Sclerosis

'Diffuse' means 'widespread'. In this condition, diffuse sclerosis occurs suddenly over large areas of the body. For example:

• Chest
• Abdomen
• Thighs
• Arms
• Legs
• Face

Also, it can affect several organs at once. That means:

• Digestive system (Digestive system - GI tract)
• Kidneys
• Heart
• Lungs

Limited Sclerosis (CREST syndrome)

Doctors often refer to Limited Scleroderma as CREST syndrome . Each letter in CREST stands for a symptom that is caused by it:

• C - Calcinosis: Excess calcium deposits in the skin. It can appear as small white bumps on the skin.
• R - Raynaud's syndrome: Discoloration and numbness of the fingertips and toes. They may turn pale, blue, or red when exposed to cold.
• E - Esophageal dysfunction: Difficulty swallowing and acid reflux.
• S - Sclerodactyly: Tightness of the skin on the fingers, making it difficult to bend and straighten the fingers.
• T - Telangiectasias: Small, red or discolored spots on the skin. These may look like spider webs.

Sine Sclerosis

In the case of Sine Sclerosis, the symptoms of Limited Sclerosis mentioned earlier occur, but the skin is not affected . That is, you may have some of the symptoms mentioned in CREST syndrome, but there is no visible thickening of the skin.

How common is Scleroderma?

Scleroderma is a rare disease . Experts estimate that only about 250 people per million in the United States have all three types of scleroderma. Of these, about 100,000 people have systemic sclerosis.

What are the symptoms of Scleroderma?

Some people with scleroderma may not have any symptoms in the early stages. However, for many, the main symptom is the appearance of thickened, waxy patches or streaks on the skin. In addition, there are several other common symptoms:

• Joint pain
• Stiffness , especially upon waking in the morning
• Fatigue (feeling extremely tired all the time)
• Unexplained weight loss

The other symptoms you experience (and where they affect you) depend on the type of scleroderma you have.

Symptoms of Localized Scleroderma

People with localized scleroderma usually only experience thickening of the skin . This thickening may be in one area or may occur in patches. It can affect the following areas of the skin:

• Chest
• Abdomen (the area around the stomach)
• Arms and legs (Your arms and legs)
• Hands and fingers
• Feet and toes

However, localized scleroderma, which affects internal organs, is very rare .

Symptoms of Systemic Sclerosis

Systemic sclerosis can cause a variety of symptoms. This also causes the skin to thicken, but usually in large areas and in patches. Especially on the face and hands. The thickened skin may start in the fingers and toes and then spread throughout the body. If you have Raynaud's syndrome , the fingers and toes in the affected limb may change color when exposed to cold (usually white, red, or a purplish-blue color).

Systemic sclerosis can also cause symptoms in your other organs and tissues. For example:

• Muscles: Numbness and swelling, especially in the hands and feet (the ends of your limbs).
• Joints: Stiffness, swelling, and loss of mobility.
• Lungs: Cough and difficulty breathing (dyspnea).
• Digestive tract: Dysphagia, heartburn, bloating, constipation, and diarrhea.
• Heart: Abnormal heartbeats (arrhythmia), fluid accumulation around the heart (pericardial effusion), and thickening of the heart muscle (fibrosis).
• Kidneys: Kidney failure (this can be life-threatening).
• Genitals: Erectile Dysfunction (ED) in men and vaginal dryness in women.

What causes Scleroderma?

In fact, doctors still don't know the exact cause of scleroderma.

Some studies have found that it can run in families to some extent (meaning it can be passed from parents to children), but it is so rare that there is not enough evidence to definitively prove that it is a genetic disorder .

Scleroderma Risk Factors

Anyone can develop scleroderma, but some groups are at higher risk:

• Women are about four times more likely to develop this disease than men.
• It most commonly occurs in people between the ages of 30 and 50. Scleroderma is very rare in people under the age of 30.
• Black people are more likely to develop scleroderma. They also tend to develop the disease earlier, are more likely to develop symptoms that affect the lungs, and have more severe skin symptoms.

What are the possible complications of Scleroderma?

People with scleroderma are at a higher risk of developing two other conditions: Raynaud's syndrome and Sjögren's syndrome .

Raynaud's syndrome affects the small blood vessels in your fingers and toes (your digits). People with this condition experience episodes of symptoms (sometimes called 'attacks'). This is when the blood vessels in your fingers suddenly contract too tightly. This can cause the skin in the affected fingers to turn pale, or lighter than your natural skin color. Sometimes, it can even appear bluish.

Sjögren's syndrome causes some of your body's glands to produce less moisture – most often the salivary glands in your mouth and the tear glands in your eyes. Some people with Sjögren's syndrome also experience muscle and joint pain.

Some types of scleroderma can cause serious complications . Some of them include:

• Kidney failure
• Pulmonary hypertension (high blood pressure in the arteries that carry blood from the heart to the lungs)
• Pulmonary fibrosis (scarring of lung tissue)
• Cardiovascular disease (problems affecting your heart and blood vessels)
• Congestive heart failure, a heart condition
• Immunodeficiency
• Gastrointestinal diseases (conditions that affect the way the body processes food and absorbs nutrients)
• Cancer

Some of these complications can be fatal . If you notice any new or changing symptoms, see a doctor as soon as possible. If you think you have symptoms of a heart attack, such as difficulty breathing or swallowing, call 911 (or your local emergency number) immediately, or go to a hospital emergency room.

How is Scleroderma diagnosed?

A doctor diagnoses scleroderma through a physical exam and several other tests.

You may also need to see a rheumatologist , a doctor who specializes in treating diseases of the immune system. He or she will examine you and ask about your symptoms. You should tell your doctor what your symptoms are, when they first started, and whether anything seems to make them worse.

You will also need to have several tests done to make sure you don't have other conditions that are causing similar symptoms.

What tests do doctors use to diagnose Scleroderma?

Diagnosing scleroderma is usually part of a process called 'differential diagnosis.' This means that your doctor will likely rule out other conditions and perform a number of tests to find out what is causing your symptoms before diagnosing you with scleroderma. Here are some of the tests you may have:

• Blood tests to see how your immune system is working.
• Pulmonary function tests to see if your lungs or respiratory system are affected.
• A biopsy is a procedure in which a small sample of the affected skin or other tissue is taken and tested in a laboratory.
• If you have digestive (GI) symptoms, an endoscopy is a test that looks down your throat or stomach with a small camera attached to a long, thin tube.

In addition, you will need to have several imaging tests to take pictures of the inside of your body. These include:

• Electrocardiogram (ECG)
• Echocardiogram (Echocardiogram - Echo)
• A chest X-ray
• A CT scan (Computed Tomography - CT scan)

How is Scleroderma treated?

There is no cure for scleroderma , but your doctor can help you find a combination of treatments that can help control your symptoms and minimize their impact on your daily life.

The type of treatment you need depends on where your symptoms are and how severe they are. Here are some common treatments for scleroderma:

• Skin treatments: You will need to use creams and moisturizers to stop your skin from drying out.
• Immunosuppressants: These drugs stop your immune system from damaging healthy cells and tissues.
• Medications to control specific symptoms: For example, you may need to take medications to control your blood pressure, make breathing easier, manage kidney failure, or relieve digestive symptoms.
• Physical therapy: A physical therapist can help you improve your physical movements.
• Phototherapy: This involves using bright, focused ultraviolet (UV) light to treat skin conditions. This can help treat thickened skin.
• Stem cell transplants: Some people with severe symptoms may need a stem cell transplant. This involves replacing damaged blood cells with healthy donor cells.

If I have Scleroderma, what should I expect?

You should expect to manage scleroderma and its symptoms for the rest of your life . Although there is no permanent cure, many people find treatments and lifestyle changes that can minimize the impact their symptoms have on their daily lives.

Living with a long-term illness can be very frustrating at times. Ask your doctor about support groups or educational opportunities to help you manage your stress and mental health.

Can Scleroderma be prevented?

Because the exact cause is unknown, there is no way to prevent scleroderma .

How do I take care of myself? (Self-care)

In addition to your usual treatments, you may be able to manage some symptoms by making some changes to your daily lifestyle. Some of these include:

• Following a healthy diet and exercise plan that suits you.
• Avoiding intense physical activity when you are not feeling well.
• Protect your skin by dressing appropriately for your environment and applying a good quality sunscreen when you go outside.
• Visit a dentist (dental care provider) for regular teeth cleaning and checkups.

When should I see my doctor?

Scleroderma can cause a variety of symptoms, so it can sometimes be difficult to recognize at first. If you have new pain or other symptoms, especially if they get worse, see a doctor. Even if your symptoms are caused by something else, your doctor can identify the cause and recommend treatments to control them.

If your scleroderma treatment doesn't seem to be working well, or if your symptoms change or get worse – especially if they affect your ability to breathe or swallow – talk to your doctor.

When should I go to the Emergency Treatment Unit (ETU) ?

If you have heart attack symptoms , such as chest pain, difficulty breathing, or if you feel like you can't swallow, call 911 (or your local emergency number) immediately, or go to a hospital emergency department.

What questions should I ask my doctor?

Don't forget to ask these questions when you see the doctor:

• Do I have Scleroderma or another disease?
• What type of Scleroderma do I have?
• What kind of treatment do I need?
• What complications should I be especially aware of?

What is the life expectancy of someone with Scleroderma?

Most people with scleroderma do not develop life-threatening complications .

However, if you have systemic sclerosis, which affects internal organs, you are at higher risk of serious complications. Your doctor will tell you what to expect and whether you are at higher risk of fatal complications.

Is Scleroderma a serious disease?

Like any chronic illness, scleroderma is a significant, serious condition . This is because you will likely have to deal with the symptoms for the rest of your life. However, that doesn't mean that scleroderma will rule your life. Everyone's experience is different. Talk to your doctor about any fears, concerns, or worries you may have.

Scleroderma can be a very frustrating condition to live with. Because doctors aren't sure what causes it and there's no one-size-fits-all treatment, it can take some time to find a treatment that will control your symptoms well. Your doctor will help you every step of the way.

Trust your gut feeling, and trust what feels 'not right'. Even a small change in symptoms could be a sign of a problem that needs to be checked out by your doctor. Don't be afraid to ask questions and share your thoughts about your symptoms or treatment.

Living with a chronic illness can be very tiring. Take time to give yourself some respect and recognition for the hard work you put into managing your symptoms.

Some important things you should remember (Take-Home Message)

Okay, we've talked a lot about Scleroderma now. In conclusion, here are the most important things you need to remember:

• Scleroderma is a rare, autoimmune disease in which the body produces too much collagen, causing the skin and sometimes internal organs to thicken.
• There are two main types of this: localized and systemic. The systemic type is a bit more dangerous because it can also affect internal organs.
• Symptoms vary. The main ones include thickening of the skin, joint pain, fatigue, and Raynaud's syndrome.
• Although there is no permanent cure, there are effective treatments to control symptoms. Talk to your doctor and develop a treatment plan that works for you.
• Early diagnosis and treatment are very important, so if you have any of these symptoms, be sure to seek medical advice.
• Since this is a long-term condition, it is important to stay mentally strong. If necessary, seek help from support groups.

Don't worry, you're not alone. With the right knowledge and medical advice, you can live successfully with this condition.

` Scleroderma, Scleroderma, Skin thickening, Immune diseases, Collagen, Raynaud's syndrome, Systemic sclerosis, Skin diseases