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Scleroderma

CREST Syndrome

A less severe form of scleroderma is called CREST.

The CREST syndrome represents symptoms including calcium skin deposits; Raynaud's phenomenon (a condition in which the blood vessels of the fingers and toes go into spasm when triggered by factors such as cold, stress, or illness; the result is cold, painful, or numb fingers and toes which in severe cases may become gangrenous); esophageal dysfunction (problems with the esophagus, the tube between the mouth and the stomach); sclerodactyly (skin damage on fingers); and telangiectasia (spider veins), limits skin damage to the fingers.

However, this disease, when coupled with pulmonary hypertension (elevated blood pressures within the lungs), can lead to heart and respiratory failure.

What is scleroderma?

There are two forms of scleroderma: localized scleroderma and systemic sclerosis. Localized scleroderma can be seen more frequently in children than the systemic form. It may involve patches of the skin on the trunk, arms, legs, or head. Other names for the localized form are morphea and linear scleroderma.

Systemic sclerosis is a chronic, degenerative disease that affects the joints, skin, and internal organs. Scleroderma is also associated with blood vessel abnormalities. Systemic sclerosis occurs only rarely in children.

Scleroderma is considered to be a multifactorial condition. Multifactorial inheritance means that "many factors" are involved in causing a health problem. The factors are usually both genetic and environmental, where a combination of genes from both parents, in addition to unknown environmental factors, produce the trait or condition. Often one gender (either males or females) is affected more frequently than the other in multifactorial traits. Females are affected with scleroderma three to four times more often than males.

What are the symptoms of scleroderma?

Scleroderma can lead to scarring of the skin, joints, and other internal organs. The following are the most common symptoms of scleroderma. However, each child may experience symptoms differently. Symptoms of systemic sclerosis may include:

Symptoms of localized scleroderma may include:

Symptoms of scleroderma may resemble other medical conditions or problems. Always consult your child's physician for a diagnosis.

How is scleroderma diagnosed?

Diagnosis of scleroderma is usually based on the changes in the skin and internal organs. An antibody test may help distinguish the type of scleroderma present. In addition to a complete medical history and physical examination, diagnostic procedures for scleroderma may include:

Treatment for scleroderma:

Specific treatment for scleroderma will be determined by your child's physician based on:

Treatment may include:

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