Scleroderma Symptoms

Common Scleroderma Symptoms

Common symptoms and signs of scleroderma are:

• Puffy or swollen fingers

• Tight, thick or hard skin

• Fingers or toes turning white, blue or red in cold weather or during stress

• Joint pain, stiffness or reduced movement

• Heartburn or difficulty swallowing

• Small red blood vessels are visible on the skin

• Calcium deposits under the skin

• Fatigue

Some people with scleroderma mainly have skin changes, while others, particularly those with systemic sclerosis, may develop symptoms involving the digestive system, lungs, heart, kidneys or blood vessels.

Scleroderma Symptoms That Should Not Be Ignored

The table below shows scleroderma symptoms linked to specific organs and when they may need medical assessment:

These symptoms do not confirm that a particular organ has been affected. They indicate that further medical assessment may be needed to identify the underlying cause.

Why Do Scleroderma Occur?

Scleroderma usually occurs due to changes in the immune system, small blood vessels and connective tissue. In affected tissues, fibroblasts can produce excess collagen, leading to fibrosis and causing the skin or other tissues to become thicker, firmer and less flexible.

The excess collagen and resulting fibrosis help explain why scleroderma can cause hard or tight skin. When the skin becomes tight around the fingers or joints, movement may become more difficult. Affected skin may also become shiny, dry or itchy, while hair growth and sweating can decrease in some areas.

Changes in small blood vessels can cause the colour and temperature changes seen in Raynaud’s phenomenon. Exposure to cold or emotional stress can cause the small blood vessels in the fingers or toes to narrow. Reduced blood flow can make the affected areas feel cold or numb and cause the skin to become pale or blue. As circulation returns, the skin may become red and feel warm, painful or tingly.

Severe or repeated reduction in blood flow can result in fingertip sores or ulcers. In systemic sclerosis, changes in blood vessels and fibrosis can also affect internal organs. Lung involvement may cause breathlessness or a dry cough, while oesophageal involvement can contribute to difficulty swallowing and acid reflux.

The exact combination of symptoms differs from person to person. Localised scleroderma generally affects the skin and tissues underneath it, whereas systemic sclerosis can affect the skin, blood vessels and internal organs. The exact cause of the immune-system changes that lead to scleroderma is not fully understood.

Scleroderma Symptoms That Require Immediate Medical Attention

Certain symptoms can signal a serious complication of systemic sclerosis and require urgent medical assessment. Seek immediate medical care for:

• A sudden marked rise in blood pressure with a severe headache, breathlessness or other new symptoms: This can occur with scleroderma renal crisis, a serious complication that can cause a rapid decline in kidney function.

• Severe or rapidly worsening breathlessness: This may indicate serious lung or heart involvement and requires urgent assessment.

• Chest pain, fainting or severe palpitations: These symptoms can occur with significant heart involvement or abnormal heart rhythms and need prompt evaluation.

• A finger or toe that becomes extremely painful, very cold, blue or dark: Severe reduction in blood flow can put the affected tissue at risk.

• Confusion, seizures or loss of consciousness: These are emergency symptoms and require immediate medical attention regardless of the underlying cause.

Symptoms of Scleroderma Based on Type

Scleroderma includes localised forms such as morphoea and linear scleroderma, as well as systemic sclerosis. The table below explains the typical symptom patterns associated with the main recognised forms.

Symptoms of Scleroderma in Children

In children, scleroderma commonly appears as localised patches or lines of hardened skin. In linear scleroderma, these changes can extend into deeper tissues such as muscle and bone. It can lead to impact movement, limb growth or nearby structures.

Symptoms of linear scleroderma may include:

• A firm line or groove in the skin

• Skin discolouration

• Hair loss in affected areas of the scalp or forehead

• Restricted movement when the affected area crosses a joint

• Differences in the length or size of an affected limb due to impaired growth

Lesions involving the head or face may sometimes be associated with deeper tissue, eye or neurological involvement and therefore require medical assessment.

Children may also get infected with systemic sclerosis and develop symptoms including:

• Puffy fingers and Raynaud’s phenomenon

• Tight or hardened skin

• Joint stiffness or other joint symptoms

• Digestive symptoms, such as difficulty swallowing or acid reflux

• Lung or heart-related symptoms

Can You Have Scleroderma Without Symptoms?

Some internal-organ changes can develop before a person notices obvious symptoms, particularly in systemic sclerosis. However, completely asymptomatic scleroderma is extremely rare, as early vascular signs (most notably Raynaud's phenomenon) are almost always present before other features appear. For this reason, doctors may use targeted assessments (such as nailfold capillaroscopy, autoantibody panels, pulmonary function testing, and echocardiography) to look for organ involvement even when symptoms are mild or absent.

Pulmonary arterial hypertension is one example. Changes in pressure within the blood vessels of the lungs may develop before noticeable breathlessness occurs. Regular screening allows doctors to detect these changes early.

Systemic sclerosis sine scleroderma is another example. This subtype causes internal organ and vascular complications in the total absence of skin thickening. Therefore, the absence of hard or thickened skin does not completely rule out systemic disease.

While early or subtle signs like cold-sensitive fingers are almost always present, internal organ changes can silently progress. Diagnosis depends on the overall clinical picture, blood tests, and specialised imaging by a healthcare professional.

When Can Scleroderma Symptoms Become Life-Threatening?

Scleroderma can become life-threatening when systemic sclerosis seriously affects the kidneys, lungs, pulmonary blood vessels or heart. The risk depends on the organs involved and the severity of the disease.

• Scleroderma renal crisis: A sudden rise in blood pressure can occur with rapidly worsening kidney function. This is a serious complication that requires urgent medical treatment.

• Severe interstitial lung disease: Scarring can make the lungs less able to expand and transfer oxygen efficiently, leading to worsening breathlessness and reduced lung function.

• Severe pulmonary atrial hypertension: Increased pressure in the pulmonary arteries can place strain on the right side of the heart. Breathlessness, fatigue, dizziness, fainting and swelling of the legs or ankles can occur.

• Serious heart involvement: Systemic sclerosis can affect the heart muscle and its electrical system. Abnormal heart rhythms, reduced heart function, chest pain, fainting or worsening breathlessness may occur.

These complications do not occur in everyone with scleroderma. The type of scleroderma, organs involved and individual disease patterns all influence the symptoms and risks.

How are Scleroderma Symptoms Diagnosed?

There is no single test that confirms scleroderma. Doctors consider the symptoms and physical examination, along with blood tests and other investigations. Additional tests may be used to assess suspected involvement of the lungs, heart, kidneys or digestive system.

The table below explains the main assessments and investigations that may be used to evaluate scleroderma symptoms and check for organ involvement.

The choice of tests depends on the symptoms, examination findings and suspected type of scleroderma. Not every person with scleroderma needs every investigation listed above. NIAMS notes that doctors use a combination of medical history, examination, laboratory tests and additional investigations because there is no single test for scleroderma.