Scleroderma

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

If you have scleroderma, your immune system triggers your body’s cells to produce too much collagen. 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 it 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). Visit a healthcare provider if you’re experiencing symptoms like pain and stiffness in your joints, especially if you notice any patches of thickened skin.

Symptoms of scleroderma

Some people with early scleroderma don’t have any symptoms. The most common symptoms include:

• Patches or streaks of thickened, waxy skin
• Joint pain
• Stiffness, especially first thing in the morning
• Fatigue
• Unexplained weight loss
• Raynaud’s syndrome
• Heartburn or acid reflux

Which other symptoms you experience depends on which type of scleroderma you have.

Types of scleroderma

Healthcare providers classify scleroderma into two main types:

Localized scleroderma

This only affects one part of your body (usually your skin). It causes thick patches or streaks on your skin that feel waxy. It’s most common on your:

• Chest
• Belly
• Arms
• Legs
• Hands
• Feet

Localized scleroderma may get better on its own. It usually doesn’t spread to other parts of your body.

Systemic sclerosis

Systemic sclerosis can affect other organs along with your skin, including your lungs, heart, stomach and small bowel. It can affect your ability to breathe or process nutrition.

Systemic sclerosis has three subtypes:

• Diffuse sclerosis: This causes thickening in multiple large areas of your skin at the same time. It’s more likely to affect your face. You may have thickened skin on your neck, upper arms, upper legs, chest or belly. It can also affect multiple organs, like your digestive system, kidneys, heart and 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. Symptoms include Calcinosis (calcium deposits in your skin), Raynaud’s syndrome, Esophageal dysfunction (trouble swallowing and acid reflux), Sclerodactyly (tight skin on your fingers) and Telangiectasis (red or discolored spots on your skin). Skin thickening is most common on your hands, forearms, feet and lower legs.
• Sine sclerosis: This causes limited sclerosis symptoms without affecting your skin. You may experience CREST syndrome symptoms, but won’t have any thickened skin.

Systemic sclerosis can cause symptoms that may seem unrelated to each other. You might notice issues like:

• Muscle numbness or tingling
• Coughing or shortness of breath
• Trouble swallowing
• Heartburn, bloating, constipation, diarrhea or trouble controlling your poop
• Abnormal heartbeats, including heart blocks
• Erectile dysfunction or vaginal dryness
• Dry eyes and mouth
• Itching

Scleroderma causes

Experts don’t know for sure what causes scleroderma. But they do know it’s an autoimmune disease. It happens when your immune system attacks healthy cells by mistake. This leads your body to produce the extra collagen that causes symptoms.

Scleroderma can run in families. Biological parents can pass it on to their children. But this is rare enough that there’s no definite proof it’s a genetic disorder.

Risk factors

Anyone can develop scleroderma. But you may have a higher risk if you are:

• Female
• Between the ages of 30 and 50
• Black

People who are Black are much more likely to have scleroderma. It’s also more common for people who are Black to develop it earlier in life, experience more severe skin symptoms and have lung symptoms.

Complications of scleroderma

If you have scleroderma, you’re more likely to have Raynaud’s syndrome and Sjögren’s syndrome.

Some types of scleroderma can cause severe complications, including:

• Kidney failure
• Pulmonary hypertension
• Pulmonary fibrosis
• Cardiovascular disease
• Congestive heart failure
• Needing a finger or toe removed
• A weakened immune system
• Gastrointestinal diseases
• Cancer

How doctors diagnose scleroderma

A healthcare provider will diagnose scleroderma with a physical exam and tests.

You might need to visit a rheumatologist, a healthcare provider who specializes in treating autoimmune disorders. They’ll examine your body and ask you about your health history. Tell your provider which symptoms you’re experiencing, when you first noticed them and if anything seems to make them worse.

Diagnosing scleroderma is usually part of a differential diagnosis. This means your provider will probably use a few tests to rule out other conditions that can cause similar symptoms before diagnosing you with scleroderma. You might need:

• Blood tests to see how well your immune system is working
• Pulmonary function tests to show if your lungs or respiratory system are affected
• A biopsy to remove a sample of your affected skin or other tissue for testing in a lab
• Endoscopy if you’re experiencing gastrointestinal symptoms

You’ll probably also need a few imaging tests, like:

• Electrocardiogram (EKG/ECG)
• Echocardiogram
• Chest X-ray
• CT scan

Scleroderma treatments

Your healthcare provider will help you find treatments that manage the symptoms and minimize how much they impact your daily routine. But there’s no cure for scleroderma.

Which treatments you’ll need depends on where you’re experiencing symptoms and their severity. Some options include:

• Skin treatments: You might need creams and moisturizers to prevent your skin from drying out.
• Immunosuppressants: These medications stop your immune system from damaging healthy cells and tissues.
• Medicines to manage specific symptoms: For example, you might need medication to manage your blood pressure, improve your breathing, treat Raynaud’s syndrome symptoms, manage kidney failure or relieve gastrointestinal symptoms.
• Physical therapy: A physical therapist will give you a customized plan of exercises and stretches to help manage pain and other physical symptoms.
• Light therapy (phototherapy): Light therapy uses bright, focused UV light to treat skin conditions. It can help treat thickened skin.
• Stem cell transplants: You might need a stem cell transplant if you have severe symptoms. It will help your body replace damaged blood cells with healthy donor cells.

When should I see my healthcare provider?

Scleroderma can cause so many different symptoms that it’s sometimes hard to notice at first. Visit a healthcare provider if you notice any new pain or other symptoms, especially if they’re getting worse.

Talk to your provider if you feel like your scleroderma treatments aren’t working as well as they used to. Or if the symptoms are changing.

Call 911 (or your local emergency number) or go to the emergency room if you’re experiencing heart attack symptoms, like chest pain, trouble breathing or you feel like you can’t swallow.

What can I expect if I have scleroderma?

You should expect to manage scleroderma and its symptoms for the rest of your life. Even though there isn’t a cure, most people find treatments to minimize how much it impacts their day-to-day life.

Living with a chronic condition can be extremely frustrating. Ask your healthcare provider about additional resources, like support groups or educational opportunities, to help you manage stress and your mental health.