Scleroderma

Scleroderma is a rare condition that makes your body produce tissue that’s thicker than it should be. Scleroderma usually affects your skin, but can cause symptoms in any tissue throughout your body.

Scleroderma is an autoimmune disorder. Autoimmune disorders happen when your immune system accidentally attacks your body instead of protecting it. Experts don’t know why your immune system turns on you. It’s like it can no longer tell the difference between what’s healthy and what’s not — between what’s you and what’s an invader like bacteria or a virus.

If you have scleroderma, your immune system triggers your body’s cells to produce too much collagen (a protein). Your body needs collagen to have strong, healthy connective tissue to support your organs and hold parts of your body in place. But when you produce too much of it, your skin and other tissue can be thicker and more fibrous than they should be.

Scleroderma is a chronic condition, which means you’ll need to manage your symptoms for a long time (maybe the rest of your life). It can also cause life-threatening complications if it affects tissue in your organs. Call 911 (or your local emergency services number) or go to the emergency room if you feel like you’re having a heart attack, can’t breathe or can’t swallow.

Visit a healthcare provider if you’re experiencing symptoms like pain and stiffness in your joints, especially if you notice thickened skin around your fingers and toes.

Types of scleroderma

Healthcare providers classify scleroderma into two main types:

Localized scleroderma: Localized means concentrated in one area. Localized scleroderma only affects one part of your body (usually your skin). It causes thick patches or streaks on your skin that feel waxy. Localized scleroderma can get better (resolve) on its own. It usually doesn’t spread to other parts of your body.

Systemic sclerosis: Systemic sclerosis can affect other organs, in addition to your skin. It can affect parts of your respiratory system (the organs that help you breathe and smell) and your digestive system (the organs that help you turn foods and drinks into energy). Scleroderma is more likely to cause serious complications if it affects your ability to breathe or process nutrition. It can be fatal. Systemic sclerosis has three subtypes — diffuse, limited and sine sclerosis.

Diffuse sclerosis

Diffuse means spread out widely. Diffuse sclerosis causes thickened skin over larger areas at once, including your:

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

It can also affect multiple organs at once, including your:

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

Limited sclerosis (CREST syndrome)

Healthcare providers usually refer to limited scleroderma with the acronym CREST syndrome. Each letter in CREST stands for a symptom it causes:

• Calcinosis (extra calcium deposits in your skin).
• Raynaud’s syndrome (color changes and numbness in your fingertips and toes).
• Esophageal dysfunction (difficulty swallowing and acid reflux).
• Sclerodactyly (tight skin on your fingers).
• Telangiectasias (red or discolored spots on your skin).

Sine sclerosis

Sine sclerosis causes limited sclerosis symptoms, but doesn’t affect your skin. You may have any CREST syndrome symptoms but not experience any thickened skin.

How common is scleroderma?

Scleroderma is rare. Experts estimate that all types of scleroderma affect around 250 out of every 1 million people in the U.S. Around 100,000 people in the U.S. have systemic scleroderma.