What is scleroderma? 

Scleroderma is a rare autoimmune disease that triggers your immune system to make too much collagen. This results in hardened, tight skin and connective tissue that can lead to muscle and joint pain. Your symptoms could range from mild to more serious. Some types of scleroderma can damage your blood vessels and organs, including your heart, lungs, and digestive tract.

This is a complex, chronic condition, but it can be successfully treated. Penn Medicine’s autoimmune disease care experts work as a team to evaluate you using the latest diagnostic tests. Our specialists also provide you with treatment options that minimize the effects of the disease, prevent complications, and help you enjoy an active life.

Types of scleroderma 

Scleroderma might only affect your skin, or it can spread to other parts of your body.

Scleroderma symptoms and signs 

The symptoms of scleroderma disease are different for every person and depend on whether you have a mild or severe form of the condition. Your symptoms might include:

  • Thickened skin, including oval-shaped skin patches or long lines of hardened skin
  • Tight skin on your fingers (sclerodactyly) or face, small bumps under your skin (calcinosis), or red spots on your skin (telangiectasias)
  • Fingers and toes that become swollen, numb, and painful and may also turn white, blue, or red in the cold (Raynaud’s syndrome)
  • Joint pain, swelling, and stiffness
  • Shortness of breath, chest pain, dry cough, and dizziness
  • Heartburn, trouble swallowing, or digestive issues like diarrhea or constipation
  • Fatigue

Complications of scleroderma

Systemic scleroderma can make it hard to get nutrients from your food. Damage to internal organs can also lead to heart failure, lung scarring, pulmonary hypertension, kidney failure, and cancer. But treatments can help lower your risk of these health issues.

What causes scleroderma? 

Scleroderma is caused by an overreaction of your immune system, which attacks healthy tissue and causes inflammation. Your body responds by making too much collagen. Doctors don’t know why this happens, but some people with scleroderma have been exposed to silica dust and polyvinyl chloride. Hormones might play a role, since people assigned female at birth have a higher chance of having scleroderma. You’re also more likely to get scleroderma if you’re African American, if you have a parent or sibling with the disease, or if you have another autoimmune disease like systemic lupus erythematosus or polymyositis.

Diagnosing scleroderma 

Your doctor will do a thorough physical exam, looking for tight, thickened skin, including scleroderma face changes. They also look for abnormalities in your small blood vessels, heart, lungs, and digestive tract. You may need several tests that check your urine, blood, heart muscle, and skin.

Scleroderma treatment and management options 

There’s no cure for scleroderma, but medications and therapies can help suppress your immune system, treat Raynaud’s phenomenon, and manage other symptoms. Physical and occupational therapy can help reduce joint stiffness and improve movement. In rare cases of severe damage, a transplant may be an option.

Precise diagnostics and therapies for rheumatological disorders 

An accurate diagnosis means you can get treatments that will help you feel better and prevent complications. As part of a leading academic medical center, Penn Medicine’s doctors are recognized for their expertise and experience treating autoimmune diseases like scleroderma. Our rheumatologists work with dermatologists and other specialists to conduct a complete evaluation and create a treatment plan focused on your symptoms and health needs. Using the most advanced tools available, our scleroderma team provides you with the highest level of ongoing care.

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