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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.

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:

  • thickening and swelling of the tips of the fingers
  • pale and tingly fingers that may become numb when exposed to cold or when emotionally upset (called Raynaud phenomenon)
  • joint pain
  • morning stiffness
  • taut, shiny, darker skin on large areas such as the face, that may hinder movement
  • appearance of spider veins
  • calcium bumps on the fingers or other bony areas
  • grating noise as inflamed tissues move
  • frozen (immobile) fingers, wrists, or elbows due to scarring of the skin
  • sores on fingertips and knuckles
  • scarring of the esophagus, leading to heartburn and difficulty swallowing
  • scarring of the lungs, leading to shortness of breath
  • heart failure and abnormal heart rhythms
  • kidney disease

Symptoms of localized scleroderma may include:

  • shiny, thickened patches of skin
  • discolored (lighter or darker) skin
  • joint tightness

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:

  • electrocardiogram (EKG or ECG) - a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage. An EKG may be done to detect changes in the EKG pattern which may be caused by changes in the heart muscle tissue due to scleroderma.
  • echocardiogram - a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.
  • x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film. X-rays may detect changes in bone and soft tissues, the gastrointestinal tract, and the lungs caused by scleroderma.

Treatment for scleroderma:

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

  • your child's overall health and medical history
  • extent of the condition
  • your child's tolerance for specific medications, procedures, and therapies
  • expectation for the course of the disease
  • your opinion or preference

Treatment may include:

  • nonsteroidal, anti-inflammatory medications (NSAIDs) or corticosteroids (to relieve pain)
  • penicillamine (to slow the skin thickening process and delay damage to internal organs)
  • immunosuppressive medications, such as methotrexate
  • treating specific symptoms, such as heartburn and Raynaud phenomenon
  • physical therapy and exercise (to maintain muscle strength)

Click here to view the
Online Resources of Pediatric Arthritis & Other Rheumatic Diseases

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