What Is Scleroderma? Everything to Know

Medically Reviewed By Nancy Carteron, M.D., FACR
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Scleroderma, or systemic sclerosis, is an autoimmune condition that causes areas of skin to become thick and hard. In some cases, it may also affect the internal organs and blood vessels. Scleroderma is an uncommon condition. There are two main types of scleroderma: localized scleroderma and systemic scleroderma.

This guide explains more about the types of scleroderma and how to recognize symptoms. It also looks at what can cause scleroderma, how to treat the condition, when to contact a doctor, and more.

What does scleroderma look like?

View the slideshow below for scleroderma pictures.

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Scleroderma on the ankle, foot, and toes

Numstocker/Shutterstock

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Scleroderma on the hands

Numstocker/Shutterstock

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Scleroerma can affect the wrist, hand, and fingers.

Numstocker/Shutterstock

What are the types of scleroderma?

A woman is looking at her hands.
eclipse_images/Getty Images

The two main types of scleroderma are localized scleroderma and systemic scleroderma.

Localized scleroderma

Localized scleroderma is a type of scleroderma that typically affects only the skin.

Localized scleroderma does not affect internal organs.

Systemic scleroderma

Systemic scleroderma is a type of scleroderma that can affect the:

  • skin
  • muscles
  • joints
  • lungs
  • kidneys
  • blood vessels
  • heart
  • intestines
  • gastrointestinal tract

The two main types of systemic scleroderma are limited cutaneous systemic sclerosis, previously known as CREST syndrome, and diffuse cutaneous systemic scleroderma.

With limited cutaneous systemic sclerosis, skin typically thickens and becomes tighter in the fingers and toes. The condition is associated with pulmonary hypertension. Pulmonary hypertension is a condition in which the pressure in the blood vessels that lead to the heart and lungs is too high.

With diffuse cutaneous systemic scleroderma, thickening and tightening of the skin typically extends from the hands to above the wrists. The condition may involve internal organs including the kidneys, lungs, or gastrointestinal tract. You may also experience pulmonary hypertension.

What are the symptoms of scleroderma?

Symptoms of scleroderma may depend on the type of scleroderma.

Symptoms of localized scleroderma

Symptoms of localized scleroderma include:

  • morphea, which is discolored patches on the skin
  • linear scleroderma, which refers to bands or streaks of thick, hard skin, usually on the arms and legs
  • en coup de sabre, which refers to linear scleroderma on the face and forehead

Symptoms of systemic scleroderma

Symptoms of systemic scleroderma may differ depending on whether you experience limited cutaneous systemic sclerosis or diffuse cutaneous systemic scleroderma.

Limited cutaneous systemic sclerosis

Skin symptoms of limited cutaneous systemic sclerosis typically affect the fingers and toes.

The acronym of the condition’s former name – CREST syndrome – detail some of the more common symptoms of this type of sclerosis. These include:

  • calcinosis, which refers to calcium deposits in the subcutaneous layer of the skin
  • Raynaud’s phenomenon, which refers to an extreme sensitivity to the cold or stress
  • esophageal dysfunction, which refers to difficulties with swallowing as a result of scarring
  • sclerodactyly, which refers to skin tightness around the toes and fingers
  • telangiectasia, which refers to red or discolored spots you may experience on your palms, hands, forearms, face, and lips

Learn more about limited cutaneous systemic sclerosis.

Diffuse cutaneous systemic scleroderma

Skin symptoms of diffuse cutaneous systemic scleroderma typically affect the hands and extend to above the wrists. It causes skin to thicken and harden. In some cases, skin changes can also affect the whole body.

Other symptoms you may experience with diffuse cutaneous systemic scleroderma include:

Symptoms may begin suddenly and worsen quickly over a few years. The condition may then lessen, and you may see improvements in your skin.

What causes scleroderma?

Experts do not know the exact cause of scleroderma. However, there are various factors that may make you more likely to develop the condition.

These include:

  • genetics, as certain genes increase your chance of developing scleroderma
  • exposure to environmental factors such as chemicals or viruses
  • changes in your immune system such as inflammation, which may trigger your body to produce too much collagen
  • hormones, as females assigned at birth are more likely to experience scleroderma than males.

What are the treatments for scleroderma?

Treatments for scleroderma can depend on the type of scleroderma. Your doctor can explain more about the treatments they recommend.

There is no known medication that specifically targets skin tightening and hardening. Treatment focuses on helping you to manage symptoms and prevent complications.

Possible treatments for scleroderma include:

  • physical therapy to improve your ability to bend and straighten your joints
  • occupational therapy to assist with helping you to maintain your daily activities
  • anti-inflammatory drugs or immunosuppressive medications for muscle pain and weakness
  • antacid drugs such as proton pump inhibitors to treat heartburn or acid reflux
  • blood pressure medication such as ACE inhibitors for kidney disease
  • calcium channel blockers for symptoms of Raynaud’s phenomenon
  • medications such as cyclophosphamide and mycophenolate to treat interstitial lung disease
  • medications for pulmonary hypertension, such as prostacyclin-like drugs, endothelin receptor antagonists, and PDE-5 inhibitors
  • the immunosuppressive medication tocilizumab for systemic scleroderma with early lung disease
  • medications such as methotrexate and TNF inhibitors for arthritis and musculoskeletal symptoms
  • intravenous Immunoglobulin and rituximab for myositis
  • HSCT autologous hematopoetic stem-cell bone marrow transplant in severe cases

Are there any home remedies for scleroderma?

Some home remedies may help you manage symptoms and live more comfortably with scleroderma.

Steps you can take at home to ease symptoms from worsening include:

  • doing gentle exercises to increase blood supply to your skin
  • keeping the skin warm to increase blood supply and reduce symptoms of Raynaud’s phenomenon
  • gently washing the skin with a fragrance-free cleanser
  • applying a fragrance-free moisturizer to keep the skin hydrated, particularly after showering or bathing
  • using a humidifier if the air is dry
  • quitting smoking if you currently smoke, as smoking can harm the skin

When should I contact a doctor?

It is important to contact your doctor as soon as you have concerns about scleroderma.

Receiving an accurate diagnosis and beginning treatment as early as possible may help to prevent loss of motion and function in the joints.

How do doctors diagnose scleroderma?

If you have symptoms of scleroderma, your doctor may refer you to a dermatologist or rheumatologist. A dermatologist specializes in conditions affecting the skin. A rheumatologist specializes in conditions that affect the bones, muscles, and joints.

There is no individual test for diagnosing scleroderma. You may need several tests before you receive a diagnosis.

Tests that can help with reaching an accurate diagnosis include:

  • skin biopsy, which involves removing a small sample of affected skin for laboratory analysis
  • X-ray
  • lung function tests
  • CT scan
  • blood tests

Around 95% of people with scleroderma have elevated antinuclear antibodies in their blood, so this may be an indicator of scleroderma. However, it is important to note that not everybody with elevated antinuclear antibodies will develop scleroderma.

Learn more about blood tests.

How common is scleroderma?

Scleroderma is a rare condition. It affects around 300,000 people in the United States.

Certain types of scleroderma are more likely to affect females assigned at birth than males. However, linear scleroderma affects males and females more equally.

Scleroderma can occur at any age. You are more likely to develop the condition between the ages of 30–50. However, linear scleroderma typically develops before age 18.

What are the complications of scleroderma?

Complications you may experience with scleroderma may include:

  • patches of light or darkened skin
  • destruction of tissue beneath the skin
  • loss of movement in joints

Early diagnosis and the right treatment can help to reduce your risk of complications.

Can I prevent scleroderma?

Experts do not know the exact cause of scleroderma, so preventing the condition may not be possible.

However, you may be able to prevent symptoms from worsening by following your treatment plan.

Early diagnosis is essential for reducing the risk of permanent damage to the joints. It is important to contact a doctor as soon as you have concerns about scleroderma. This can help prevent permanent damage and reduce the risk of other complications.

Frequently asked questions

Here are some more frequently asked questions about scleroderma.

What are the first signs of scleroderma?

For localized scleroderma, you may notice that patches of skin begin to feel hard or thicker. This will typically occur on your arms and legs.

One of the early signs of systemic scleroderma is Raynaud’s phenomenon. You may experience increased sensitivity to the cold or to stress weeks or years before you notice thickening of the skin. You may also develop puffy fingers or hands.

Is scleroderma fatal?

Although there is no cure for scleroderma, most people with the condition can continue to lead a full life. However, delaying treatment increases the risk of permanent damage to your joints. Contact your doctor as soon as you experience symptoms of scleroderma to reduce your risk of complications.

What is the difference between scleroderma and systemic sclerosis?

Scleroderma typically refers to sclerosis or fibrosis of the skin.

Systemic sclerosis, or systemic scleroderma, also affects the skin, joints, muscles, and bones. However, it can also affect the internal organs.

Summary

Scleroderma is a condition that causes thickened, tightened skin. The two main types of scleroderma are localized scleroderma and systemic scleroderma.

Localized scleroderma typically affects the arms and legs. Systemic scleroderma tends to affect the skin on the toes and fingers, as well as the hands and above the wrists.

Contact your doctor as soon as you have concerns about scleroderma. Receiving an accurate diagnosis as early as possible will help to ensure you can begin the right treatment and reduce your risk of complications.

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Medical Reviewer: Nancy Carteron, M.D., FACR
Last Review Date: 2022 Oct 4
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