What is scleroderma?

Scleroderma is hardening of the skin. It is a rare autoimmune rheumatic disease that causes inflammation in the skin and connective tissues including the tendons and cartilage. It can affect the entire body (systemic) or only one area (localized). Localized scleroderma tends to be milder and usually does not affect the internal organs. Systemic scleroderma may be limited or diffuse. Diffuse scleroderma is the most serious form which damages blood vessels and major internal organs including the heart, lungs and kidneys.

Autoimmune diseases occur when the immune system overreacts and attacks healthy tissue causing inflammation. The inflammation triggers development and build up of excessive collagen in the skin and organs which makes them hard and tight.

Onset of the disease may be gradual or rapid. Gradual onset affects the skin on the fingers, hands, face, lower arms and legs below the knees. Rapid onset affects the fingers and toes, progresses to elbows and knees, and then to upper arms, trunk and thighs, usually with some internal organ damage.

What are the symptoms of scleroderma?

Symptoms vary from person to person and can be mild or life threatening depending on the organs involved. Symptoms also vary depending on the type of scleroderma.

Localized scleroderma symptoms

Localized scleroderma is the type found in young children.

It is benign and frequently self-limiting. Changes are limited to a few areas of the skin or muscle and rarely spread. The localized form is also called Morphea, which is patches of waxy thick, tight skin on the fingers vary in shape, size and color. The patches can grow or shrink and may disappear naturally.

Limited scleroderma symptoms include:

  • Linear scleroderma which begins as a streak of hardened waxy skin on an arm or leg or the forehead and involves deeper layers of the skin and can affect the joints.

Systemic Scleroderma symptoms:

Symptoms involve the skin, blood vessels, muscles, joints, esophagus, gastrointestinal tract, lungs, kidneys, heart and other internal organs. The affected tissues become hard (sclerosis) and interfere with function. Symptoms include:

  • Hardened skin and skin ulcers
  • Calcium deposits in connective tissues
  • Raynaud’s Phenomenon where small blood vessels shut off circulation in response to cold turning the fingers and toes blue or purple.
  • Esophageal dysfunction including swallowing problems
  • Sjogren syndrome (dry mouth and eyes)
  • Telangiectasia is tiny, swollen blood vessels that appear as red spots on the hands and face
  • Skin thickening may spread to the rest of the body and can damage the lungs, kidneys, and heart

What causes scleroderma?

The cause is unknown but several factors that increase the risk. Scleroderma is not inherited but if a close relative has it, that increases the risk. Women are affected 4 times more often than men. The adult age at onset is usually between 25 and 55, but children are diagnosed between ages 2 and 14. All races and ethnicities are at risk, but scleroderma is more common and severe in African Americans.

How is Scleroderma diagnosed?

Your Ortho Illinois rheumatologist will review your medical history and symptoms, conduct physical, order lab tests, and x-rays. If internal organ involvement is suspected your doctor will order a CT scan. Specialists may order additional testing.

What are the treatment options for scleroderma?

Treatment options depends on type of scleroderma, your symptoms and the tissues and organs affected. There is no cure. The goal of treatment is to control symptoms and complications with medications, physical and occupational therapy.

Anti-inflammatory medications can manage pain and reduce swelling. Topical steroid creams can improve skin changes including tightness and itching. Oral, injectable or IV steroids can help to manage joint pain and inflammation. Immunosuppressants can help to tamp down the immune response to control symptoms. Vasodilators can help improve blood flow by widening blood vessels. Physical and occupational therapy can help to relieve pain, improve muscle strength and mobility and help you accommodate to daily activities.

Ortho Illinois has a team of rheumatologists and specialists like orthopedists, who will work together with cardiologists, nephrologists, and pulmonologists to provide you with treatment options based on your specific case.