What Is Localized Scleroderma?

Scleroderma is a rare disease affecting about 300,000 people in the United States, according to the Scleroderma Foundation. It has two forms: localized and systemic. Scleroderma manifests itself differently in every patient---one may have mild symptoms, with only skin changes, while another patient may be disabled by the scleroderma because it involves organs such as the kidney, lungs and blood vessels.

What is Scleroderma?

• Scleroderma is a chronic autoimmune disorder involving the connective tissues. People with autoimmune disorders have immune systems that cannot distinguish between healthy and harmful substances, according to MedlinePlus. With scleroderma, the immune system damages normal tissues and causes scar tissue to develop in various organs, writes Dr. William C. Shiel Jr. The affected organs harden and thicken so that they do not function normally anymore.

Localized Scleroderma

• Localized scleroderma is considered the milder form of the disease; it involves limited parts of the body such as the skin and muscles. The localized form affects children more often, and the systemic form is more common in adults. Localized scleroderma is divided into two groups: morphea and linear scleroderma. Morphea sufferers get patches of waxy skin that vary in size, shape and color. The patches may change in size and disappear randomly. Linear scleroderma starts out as a hardened line on the forehead, arm or leg and causes thickening in the underlying layers of skin and joints, according to the Scleroderma Foundation. The thickening may decrease mobility, and in children this can lead to problems in limb growth.

Systemic Scleroderma

• Systemic scleroderma involves multiple parts of the body. It can cause hardening, thickening and scarring in major organs such as the lungs, kidneys, heart, gastrointestinal tract and blood vessels as well as the skin and muscles. Complications and other diseases may result from the hardening and scarring of organs due to the organs not being able to function properly; possible complications include pulmonary hypertension, high blood pressure, kidney disease and various digestive system problems.

Cause

• The cause of scleroderma is still unknown, although researchers believe that genetics and the environment are possible factors. Scleroderma patients usually have family members that also have autoimmune diseases, explains Shiel. However, most people with scleroderma are the only ones in their family who have that particular disease.

Diagnosis

• It is difficult to diagnose scleroderma because it shares symptoms with other autoimmune diseases, according to the National Institute of Arthritis and Musculoskeletal and Skin Diseases. In general, a patient can expect a close review of her medical history and symptoms as well as a physical exam, lab work and skin biopsies. The physician will order labs that specifically test for autoimmunity.

Treatment

• There is no cure for either form of scleroderma, although treatments are available. Treatment plans vary from one patient to the next depending on the needs, symptoms and severity of the disease, according to Shiel. Scleroderma treatment targets the specific areas involved or particular symptoms a patient is experiencing. A common treatment is immunosuppresant therapy, or medications that suppress the immune system. Some patients may not require treatment at all.