Systemic Sclerosis

What is Scleroderma?

Scleroderma, which means “hard skin,” affects about 300,000 Americans. It is not contagious, infectious or cancerous. Scleroderma refers to two conditions - localized scleroderma and systemic sclerosis.

Localized Scleroderma

This condition mainly affects the skin, but also can affect tissues beneath the skin including fat, connective tissue, muscle and bone. Localized scleroderma is more common in children. In adults, it affects women more often than men. It can appear between the ages of 20 and 50.

There are two types of localized scleroderma:

• Linear. This type causes lines or streaks of thickened skin form over an area of the body, such as an arm, leg or the head, and may cause deep-tissue injury (ulcers on the skin).
• Morphea. It is characterized by one or more hard, oval-shaped, whitish or darkened patches of skin.

Systemic Sclerosis

This condition can affect many parts of the body, such as the skin, blood vessels, digestive system, heart, lungs, kidneys, muscles and joints. Thirty percent of people with scleroderma have this type. Systemic sclerosis may be classified as either limited or diffuse.

• Limited scleroderma. This kind affects the skin on the face, fingers and hands, and lower arms and legs. For many people, the first symptoms of systemic sclerosis are Raynaud’s phenomenon and puffy fingers, which can begin several years before other symptoms. If internal organs are involved, it tends to be mild. However, some people experience severe Raynaud’s phenomenon, gastrointestinal problems or serious effects on the lungs. Some patients with limited scleroderma also have CREST syndrome. CREST is an acronym using the first letter of five conditions: calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia.
• Diffuse scleroderma. Skin thickening is widespread. It may affect any part of the body, especially the hands, arms, thighs, chest, abdomen and face. Itching, decreased flexibility and pain can also occur. Diffuse scleroderma may affect the blood vessels, heart, joints, muscles, esophagus, intestines and lungs. The severity of internal organ involvement varies. Kidney problems may lead to high blood pressure and, if untreated, kidney failure. Lung damage is the leading cause of death with this condition. In rare cases, systemic sclerosis may involve internal organs but not the skin. This condition is known as systemic sclerosis sine scleroderma.

Scleroderma Causes

The cause of scleroderma is unknown. However, it is believed to be an autoimmune disease, meaning it occurs when the body’s immune system mistakenly attacks the its own cells and tissues. Scientists know that people with scleroderma overproduce collagen, a key component of connective tissue. Too much collagen causes the skin to thicken and may cause internal organs to function abnormally.

Scleroderma Symptoms

Symptoms of scleroderma can vary and depend in part on which form you have. They include:

• Calcium lumps under the skin (calcinosis), which occasionally may break through the skin and leak a chalky white liquid
• Digestive problems, including decreased motion (dysmotility) in the esophagus, which may give the sensation of food sticking in the throat; acid reflux and constipation
• Dryness of the mouth, eyes, skin or vagina caused by decreased secretions from moisture-producing glands
• Heart problems, including irregular heart rhythms, heart failure, inflammation of the lining of the heart (pericarditis) or fluid around the heart
• Kidney problems, which can cause severe high blood pressure and, potentially, kidney failure
• Lung problems, including scarring of the lung tissue or high blood pressure in the arteries of the lungs (pulmonary arterial hypertension)
• Joints that are stiff, swollen, warm, or tender
• Muscle weakness, often in the upper arms or thighs
• Raynaud’s phenomenon, a condition in which blood flow to the skin decreases in response to cold temperatures or emotional stress, causing fingers or toes to turn blue
• Thickening of the skin on the fingers (sclerodactyly), which makes them difficult to bend or straighten
• Skin changes, such as hardening and thickening, and a shiny appearance or loss of hair over the affected area
• Telangiectasia, small dilated blood vessels that show through the skin

Scleroderma Diagnosis

Doctors diagnose scleroderma by asking questions about the person's medical history, performing a physical examination and ordering laboratory tests and a skin biopsy (a procedure in which a sample of skin tissue examined under a microscope).

Scleroderma Treatment

There is no cure, but treatments are available to help manage symptoms. They include medications and lifestyle modifications. The symptoms of the disease can vary greatly one person to another, so treatments options are very broad. A rheumatologist will likely be the primary doctor leading the healthcare team. Other specialists may be needed to treat heart, kidney, skin, lung, dental and gastrointestinal problems that occur with scleroderma.

Scleroderma Self Care

Taking a proactive role in treatment is one of the best ways to help manage a person's health. This process is called self management.