What Is Scleroderma? When Skin Tightens and Organs Are Affected
Scleroderma is a rare condition in which the body’s immune system attacks its own healthy tissues. First, it typically causes the skin to become stiff and tight. Next, it can harm organs such as the lungs, heart, and kidneys. In this article, we will explore what scleroderma is, how it affects people, and what doctors do to treat it.
What Is Scleroderma?
Scleroderma comes from two Greek words that mean “hard skin.” It belongs to a group of diseases called connective tissue disorders.1 In connective tissue disorders, the proteins that support skin and organs thicken. As a result, tissues lose their normal flexibility. Over time, this change can limit movement and blood flow. In addition, it can damage organs.
Scleroderma ranges from mild to severe. In mild cases, only patches of skin harden. In severe cases, organs such as the lungs or heart become involved.2 No two people have exactly the same course. However, most form early warning signs on their hands and face.
How Does Scleroderma Affect the Skin?
In the early stages, skin changes often appear in the fingers. They may swell and feel tight. Next, they may turn shiny and lose normal lines. Cold temperatures or stress can trigger blood vessel spasms called Raynaud’s phenomenon. During these spasms, fingers or toes turn white, then blue, and finally red as blood rushes back in.3
Skin can thicken or harden over time. Small bumps or patches of hardened tissue may show up on the chest, back, or face. These areas may feel firm to the touch. In some people, the skin grows too tight around joints. This change can limit the ability to bend elbows, knees, or fingers fully.
How Does Scleroderma Affect Organs?
Not all forms of scleroderma involve organs. However, in those that do, serious problems can develop.4 For example:
- Lungs: Scar tissue can form in the lung tissue. This change, called pulmonary fibrosis, makes it hard to breathe.
- Heart: The heart muscle can stiffen. As a result, it may pump less effectively. In some cases, fluid buildup around the heart may occur.
- Kidneys: Blood vessels in the kidneys may narrow suddenly. This event, called scleroderma renal crisis, can cause high blood pressure and kidney failure if not treated quickly.
- Digestive Tract: Tissue can thicken in the esophagus and intestines. This change may cause heartburn, difficulty swallowing, and poor nutrient absorption.
Each affected organ brings its own set of symptoms. Often, these signs develop slowly, over months or years. That is why doctors monitor people with scleroderma closely.
What Causes Scleroderma?
Researchers believe scleroderma happens when the immune system attacks healthy cells. As a result, the body overproduces collagen, a protein that builds connective tissue. This extra collagen typically causes tissues to thicken and harden.5
Experts do not know what triggers this immune response. Genetics may play a role, since scleroderma can run in families. Environmental factors, such as certain chemicals or viral infections, may also contribute. However, no single cause has been proven.
Who Is at Risk?
Women develop scleroderma more often than men. Most cases begin between the ages of 30 and 50. However, younger and older people can also be affected. Certain groups, including African Americans and Native Americans, may face more severe forms.
People with other autoimmune diseases, such as lupus or rheumatoid arthritis, have a slightly higher risk. However, scleroderma can occur in anyone. Early signs include ongoing swelling in the hands and unexplained skin changes.
How Do Doctors Diagnose Scleroderma?
Diagnosing scleroderma starts with a detailed medical history and physical exam. The doctor checks the skin for thickening, looks for signs of Raynaud’s phenomenon, and feels the joints for stiffness.
Next, blood tests look for specific autoantibodies. These markers, such as anti-centromere or anti-topoisomerase I antibodies, help confirm the diagnosis. In addition, lab tests assess organ function.
Imaging tests also play a vital role. A high-resolution CT scan of the lungs can detect early fibrosis. An echocardiogram assesses heart function. Finally, a skin biopsy may show thickened collagen layers under a microscope.
When doctors catch scleroderma early, treatments can slow tissue damage and preserve organ health.
How Is Scleroderma Treated?
No cure exists for scleroderma today. However, treatments can manage symptoms and protect organs. A doctor will tailor therapy based on the type and extent of organ involvement.
- Skin Care: Moisturizers can keep skin supple. Physical or occupational therapy helps maintain joint motion.
- Blood Vessel Support: Medications such as calcium channel blockers ease Raynaud’s phenomenon by widening blood vessels.
- Immune Modulation: Drugs like methotrexate or mycophenolate mofetil suppress the overactive immune response. Biologic agents may target specific pathways.
- Organ Protection: For lung fibrosis, drugs such as nintedanib slow scar formation. High blood pressure medicines such as ACE inhibitors prevent kidney crisis.
Ongoing monitoring remains crucial. Regular tests ensure treatments work and catch new organ changes early.
How to Live with Scleroderma
Adjusting to life with scleroderma often involves teamwork. Patients collaborate with rheumatologists, dermatologists, and other specialists. In addition, good self-care habits protect health and improve quality of life.
First, skin care matters. Use gentle soaps and thick moisturizers to prevent cracking. Wear warm gloves in cold weather to avoid Raynaud’s attacks. Next, stay active. Gentle stretching and low-impact exercise maintain flexibility and lung capacity.
Moreover, nutrition plays an important role. Small, frequent meals help if the digestive tract slows down. Drink plenty of fluids and avoid foods that trigger heartburn. In addition, close dental care prevents gum issues if mouth opening tightens.
Finally, build a support network. Scleroderma can affect mood and energy. Talking with others who face the same challenges can bring hope. Many groups exist both online and in local communities.
Outlook and Support
Scleroderma remains a lifelong condition for most people. However, early diagnosis and modern treatments have improved outcomes greatly in recent years. Many live active, fulfilling lives despite the diagnosis.
If you notice persistent skin changes, Raynaud’s phenomenon, or new breathing difficulties, see a doctor promptly.
