Scleroderma – Symptoms and Causes
What is Scleroderma
Scleroderma is a rare medical condition that causes the skin to become hard and tight. The name comes from Greek words meaning “hard skin.”
This disease can affect just the skin or extend to blood vessels, internal organs, and the digestive system.
There are two main types of systemic scleroderma: limited and diffuse. These types differ in how much skin is affected, but both can impact internal organs. A third type called morphea (or localized scleroderma) only affects the skin.
While doctors haven’t found a cure for scleroderma, several treatments can:
- Ease symptoms
- Slow disease progression
- Improve quality of life
People with scleroderma often need care from several specialists to manage different aspects of the disease.
Types
Morphea comes in different forms. Here are the main types:
- Plaque Morphea: The most common form. It appears as oval patches that are hard in the center.
- Generalized Morphea: Covers larger skin areas with four or more patches.
- Linear Morphea: Shows up as bands on arms, legs, or forehead, often affecting deeper tissues.
- Bullous Morphea: Rare type with fluid-filled blisters.
- Deep Morphea: Affects fat and tissue below the skin.
Some people may have more than one type at the same time. The type affects both treatment choices and how the condition might progress.
Signs and Symptoms
Skin Changes
Most people with scleroderma experience skin hardening and tightening. This typically starts in the fingers, hands, feet, and face before possibly spreading to other areas like the forearms, upper arms, chest, abdomen, and legs.
Early signs often include:
- Swelling
- Itchiness
- Skin color changes (lighter or darker)
- Shiny appearance due to tightness
Some patients develop small red spots called telangiectasia on their hands and face. Calcium deposits might form under the skin, especially at fingertips, creating bumps that X-rays can detect.
Cold Sensitivity
Many scleroderma patients experience heightened sensitivity to cold temperatures. When exposed to cold or during emotional stress, small blood vessels in fingers and toes contract more than normal. This causes:
- Pain or numbness
- Color changes (white, blue, gray, or red)
- Discomfort in affected digits
This condition can also occur in people without scleroderma.
Digestive Issues
Scleroderma can affect any part of the digestive system, from the esophagus to the rectum. Common symptoms include:
| Symptom | Description |
|---|---|
| Heartburn | Burning sensation in chest |
| Swallowing problems | Difficulty getting food down |
| Bloating | Feeling of fullness |
| Diarrhea | Loose, watery stools |
| Constipation | Difficult bowel movements |
| Bowel control issues | Inability to control bowel movements |
Breathing and Heart Problems
When scleroderma affects the heart or lungs, patients may experience:
- Shortness of breath
- Reduced exercise tolerance
- Dizziness
The disease can cause scarring in lung tissues, leading to worsening breathing problems over time. Medications may help slow this lung damage progression.
Scleroderma can also cause increased blood pressure between the heart and lungs, a condition called pulmonary hypertension. This not only causes breathing difficulties but may lead to fluid buildup in the legs, feet, and sometimes around the heart.
Causes
Scleroderma develops when the body makes too much collagen, which then builds up in tissues. Collagen is a protein that forms connective tissues, especially in the skin.
The exact cause remains unknown, but immune system dysfunction likely plays a key role. Most experts believe scleroderma results from several factors working together:
- Immune system problems: The body attacks its own tissues.
- Genetic factors: Family history may increase risk.
- Environmental triggers: Certain chemicals or viruses might set off the condition.
These factors together may prompt the overproduction of collagen that leads to hardening and scarring of tissues.
Risk Factors
Scleroderma can affect anyone, but it appears more frequently in people assigned female at birth. Most cases develop between ages 30 and 50. Black individuals often experience earlier onset, more extensive skin involvement, and higher rates of lung disease.
Several factors may increase the chance of developing scleroderma:
Genetic Factors
- Specific gene variations may increase susceptibility
- Explains why scleroderma occasionally runs in families
- May account for higher rates in certain racial and ethnic groups
Environmental Triggers
- Exposure to certain viruses
- Some medications and drugs
- Repeated contact with harmful chemicals, especially in workplace settings
- However, most people with scleroderma have no clear identified trigger
Immune System Issues
- Scleroderma is believed to be an autoimmune condition
- The body’s immune system mistakenly attacks connective tissues
- Often occurs alongside other autoimmune conditions such as:
- Rheumatoid arthritis
- Lupus
- Sjogren syndrome
Scientists continue to study how these elements interact to cause scleroderma and why some people develop the condition while others don’t.
Complications
Scleroderma can cause various complications throughout the body, ranging from mild to severe. These complications affect multiple organ systems and tissues.
Severe Raynaud’s phenomenon can damage fingertips, creating pits or sores in the skin. In some cases, finger tissue may die due to restricted blood flow.
Lung involvement often includes scarring that reduces breathing capacity and exercise tolerance.
Patients may develop pulmonary hypertension, where blood pressure rises in the arteries leading to the lungs.
Kidney complications can be serious. Scleroderma renal crisis involves sudden high blood pressure and rapid kidney failure, requiring immediate treatment to save kidney function.
Heart problems occur when scarring affects heart tissue. This can lead to:
- Irregular heartbeats
- Heart failure
- Inflammation of the heart sac
Dental issues arise as facial skin tightens, making the mouth smaller and narrower.
This creates challenges for:
- Brushing teeth
- Professional dental cleaning
- Dental restorations
Reduced saliva production further increases cavity risk.
The digestive system suffers multiple complications including heartburn and swallowing difficulties.
Many patients experience cramping, bloating, constipation, or diarrhea. Bacterial overgrowth in the intestines can interfere with nutrient absorption.
Joint function decreases as skin tightens over joints, limiting flexibility and movement. Hand function is particularly affected, making daily tasks difficult.
Early identification and management of these complications is essential to maintain quality of life and prevent permanent damage to affected organs and tissues.
