National Scleroderma Awareness Month

We've covered many autoimmune diseases in this space, and it's always remarkable how many different pathways inflammation can take. In the case of scleroderma, this rheumatic autoimmune disease tends to involve the thickening or tightening of the skin and the connective tissue under it, but many other symptoms may be present. Autoimmune diseases can be difficult to diagnose due to the variety of symptoms, and scleroderma is no exception. Due to this, research on the disease is limited and needs increased attention in order for doctors to better treat scleroderma.

The Facts About Schleroderma

Scleroderma presents itself with a variety of symptoms:

• Tightening of skin.
• Joint pain.
• Thickening and swelling of fingers.
• Reynaud's phenomenon: pale fingers that become numb or tingle when exposed to cold or stress.
• Limited mobility in the fingers, wrists, or elbows: caused by skin tightening.

The tightening of skin can appear in different ways: in small areas; as ovals or straight lines; over wider areas; or over entire limbs. The taut skin may appear dark or shiny, and it may limit movement.

An estimated 300,000 Americans have scleroderma, although the difficulty of diagnosis makes this number hard to pin down. About one-third of these patients have systemic scleroderma, the most serious form of the disease. There are two types of the disease, localized and systemic scleroderma.

Localized scleroderma

Typically only affecting the skin, localized scleroderma can spread to muscles, bones, and joints, but not to the internal organs. The following symptoms may appear:

• Morphea: discolored skin patches.
• Linear scleroderma: streaks or bands of thick, hard skin on the arms and legs.
• En coup de sabre: linear scleroderma on the face or forehead.

Systemic scleroderma

The most serious form of the disease, systemic scleroderma can affect the skin, muscles, joints, blood vessels, and internal organs including the lungs, kidneys, and heart. The following symptoms may appear:

• Calcium bumps: appear on bony areas, such as fingers, elbows, and knees.
• Sores on fingertips and knuckles.
• Noisy joints: a grating noise when attempting to move inflamed areas.
• Esophageal issues: can lead to heartburn or trouble swallowing.
• Lung scarring: can lead to shortness of breath.
• High blood pressure: can affect the kidneys.
• Heart failure.
• Abnormal heart rhythms.

Diagnosis and Treatment

As we've said, diagnosing scleroderma can be difficult because some of the symptoms are shared with other autoimmune and rheumatic disorders. Typically doctors will look for the following symptoms as part of an assessment of schleroderma:

• Reynaud's phenomenon.
• Skin thickening, swelling, and tightening.
• Telangiectasias: enlarged blood vessels on the face, hands, and around nail beds.
• Calcium deposits: most commonly on the skin.
• Joint pain.
• Shortness of breath.
• High blood pressure: especially involving kidney problems.
• Heartburn.
• Digestive tract issues: difficulty swallowing; bloating and constipation; weight loss caused by poor nutrient absorption.

Treatment and Management

Living with scleroderma is difficult, as there is no drug that has been shown to stop or reverse the hardening and thickening of skin, the major symptom of the disease. Other symptoms (such as heartburn, kidney disease, Reynaud's phenomenon, and joint and muscle pain) can be improved with drugs, but lifestyle management is one of the most common methods of treatment.

Some methods of pain and lifestyle management may include:

• Physical and occupational therapy.
• Lifestyle adaptation: to reduce the impact of physical limitations.
• Exercise: to combat stiffness.
• Heavier clothing in layers and avoiding cold: to combat stiffness.
• Dietary changes: to minimize digestion issues.
• Frequent moisturization: to prevent skin damage.
• Family and mental health support: to improve self-image caused by the impact of scleroderma.