Scleroderma is a rare autoimmune disorder characterized by progressive hardening and thickening of the skin, connective tissues, and internal organs.

It affects roughly 300,000 people in the United States, with women being four times more likely to develop it than men. Despite advances in medicine, there is currently no cure for scleroderma, but various treatment options are available to manage its symptoms and improve quality of life.

Causes and Risk Factors

The exact cause of scleroderma is unknown, but it is believed to be a combination of genetic and environmental factors that trigger an abnormal immune response against the body’s own tissues.

Some of the risk factors that can increase a person’s chances of developing scleroderma include:.

Gender: Women are four times more likely to develop scleroderma than men.
Age: Most people with scleroderma are diagnosed between the ages of 30 and 50.
Family history: Having a close relative with scleroderma increases the risk of developing it.
Environmental factors: Exposure to certain toxins, such as silica and organic solvents, may increase the risk of developing scleroderma.

Symptoms

The symptoms of scleroderma vary depending on the type and severity of the condition, but they generally fall into two categories: localized scleroderma and systemic sclerosis.

Localized Scleroderma

Localized scleroderma only affects the skin and does not involve the internal organs. There are two types of localized scleroderma:.

Morphea: This type of scleroderma involves hard, oval-shaped patches of skin that are white, yellow, or brown in color. These patches may be itchy, but they do not cause any other symptoms.
Linear scleroderma: This type of scleroderma involves a line or band of thickened, waxy skin that usually runs down an arm or leg. In some cases, it may affect the face or forehead. The skin may become tight and difficult to move, and there may be loss of hair and sweat glands in the affected area.

Systemic Sclerosis

Systemic sclerosis, also known as systemic scleroderma, can affect the skin, connective tissues, blood vessels, and internal organs, such as the lungs, heart, kidneys, and digestive tract. There are two types of systemic sclerosis:.

Limited cutaneous systemic sclerosis: This type of systemic sclerosis affects the skin on the hands, face, feet, and forearms. It may also affect the digestive tract and the blood vessels in the fingers and toes, causing Raynaud’s phenomenon.
Diffuse cutaneous systemic sclerosis: This type of systemic sclerosis affects the skin on the trunk, upper arms, and thighs, as well as the internal organs. It may cause rapid progression of skin thickening, joint pain, muscle weakness, and fatigue.

Diagnosis

Scleroderma can be difficult to diagnose, as its symptoms can mimic those of other conditions.

A diagnosis of scleroderma is typically made based on a combination of medical history, physical examination, and laboratory tests, including blood tests, imaging scans, and skin biopsies. The presence of certain antibodies, such as anti-centromere and anti-Scl-70 antibodies, may also be indicative of scleroderma.

Treatment

While there is no cure for scleroderma, various treatment options are available to manage its symptoms and improve quality of life.

Medications

Medications may be prescribed to manage the various symptoms of scleroderma, such as:.

Nonsteroidal anti-inflammatory drugs (NSAIDs) to alleviate joint and muscle pain
Corticosteroids to reduce inflammation and slow down the progression of skin thickening
Immunosuppressants to suppress the immune system and prevent further damage to the organs
Calcium channel blockers to relax the blood vessels and improve blood flow to the fingers and toes
Proton pump inhibitors to treat acid reflux and promote healing of the esophagus

Physical Therapy

Physical therapy and occupational therapy may be recommended to help maintain joint flexibility, prevent muscle wasting, and improve range of motion. Exercises that promote blood flow, such as walking and swimming, may also be beneficial.

Surgery

Surgery may be necessary in some cases of scleroderma to address complications, such as digestive tract or lung problems. For example, a lung transplant may be required if the lungs are severely affected by scleroderma.

Prevention

As the exact cause of scleroderma is unknown, there are no specific measures that can be taken to prevent it.

However, avoiding exposure to certain environmental toxins, such as silica and organic solvents, may help reduce the risk of developing scleroderma.

Conclusion

Scleroderma is a rare autoimmune disorder that can significantly impact a person’s quality of life. While there is currently no cure for scleroderma, various treatment options are available to manage its symptoms and slow down its progression.

Early diagnosis and treatment are crucial in improving outcomes for those with scleroderma.