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Diseases & Conditions

Pediatric Scleroderma

What is scleroderma?

Scleroderma means hard (sclero) skin (derma). It is a rare autoimmune disease in which normal tissues are replaced with dense, thick scar tissue. Scleroderma can affect the skin or other organ systems. In children, scleroderma most frequently affects only the skin.

What are the types of pediatric scleroderma?

There are two broad types of scleroderma:

  • Localized scleroderma – most common in children. This form of scleroderma does not change into the generalized forms of scleroderma.
  • Systemic scleroderma (sclerosis) – rare in children
Localized scleroderma

Localized scleroderma is the most common type of scleroderma in children and it usually affects the skin, only occasionally spreading to the underlying muscles.

There are two main types of localized scleroderma: linear scleroderma and morphea.

When the areas of skin affected appear in a band, it is called linear scleroderma. Linear scleroderma most often occurs on an arm or leg and occasionally on the face or neck. It can result in cosmetic problems, growth abnormalities of the affected area and if it crosses joint lines, limitation of joint motion and contractures (inability to straighten the joint).

Skin changes that appear in patches – either as a single patch or in a group – are called morphea. Morphea appears waxy and usually has an ivory or white color.

In both linear scleroderma and morphea, the skin may change to appear white with purple borders.

Systemic sclerosis

In addition to skin changes, a child with systemic sclerosis may also develop problems with internal organs (such as the kidneys, heart, lungs and gastrointestinal tract). Children with systemic sclerosis have more widespread skin changes that can result in limited joint movement.

Raynaud's phenomenon (white, blue and red color changes of the fingers and/or toes upon exposure to cold or stress) is present early in a child with systemic sclerosis, as well as fatigue, joint pain, difficulty swallowing, abdominal pain, heartburn, diarrhea and shortness of breath.

Children with systemic sclerosis should be checked often for high blood pressure, as well as lung, kidney, gastrointestinal and heart problems, to detect and treat potential internal organ involvement.

What are the signs and symptoms of systemic sclerosis?

The skin changes of systemic sclerosis can include:

  • Loss of the skin's ability to stretch
  • Light or dark discoloration of the skin
  • Thinning of the skin
  • Curling of the fingers (contractures)
  • Decreased hand function because of skin tightening on fingers and hand

Initially, the skin of the hands and feet appears swollen. Over time, the skin tightens and hardens and may appear to have ridges, depressed areas, or small pits that are seen mostly at the fingertips.

Since there is not much pain, it is common for scleroderma to be present for quite some time before a parent or child becomes concerned.

Other signs and symptoms of systemic sclerosis may include:

  • Joint inflammation with stiffness and pain
  • Raynaud's phenomenon – abnormal sensitivity to cold, which is usually seen in the hands. Signs include color changes in the hands (white, blue, red), tingling, discomfort, and decreased sensation.
  • Sores (ulcers), mostly on the finger tips
  • Digestive problems (heartburn, trouble swallowing, diarrhea, stomach cramps)
  • Itching
  • Fatigue (easily becoming tired)
  • Muscle weakness

What causes pediatric scleroderma?

The exact cause of pediatric scleroderma is unknown. It seems to involve a disorder of the cells lining small blood vessels.

Scleroderma may also be related to defects in the immune system, in which the body's normally protective defense system causes damage to its own tissues.

Normally, the immune system helps defend the body against infection. In patients with scleroderma, the immune system triggers other cells to produce too much collagen (a protein). This extra collagen is deposited in the skin and organs, which causes hardening and thickening (similar to the scarring process).

Scleroderma is not contagious, so people cannot "catch it" or pass it on to someone else.

Who is affected by pediatric scleroderma?

There are only 5,000 to 7,000 children with scleroderma in the United States. Only 1.5 percent of all people with scleroderma develop it before age 10, and 7 percent develop it between ages 10 and 19.

Two thirds of children who acquire scleroderma are female.

How is scleroderma diagnosed?

The diagnosis of scleroderma is often made by a rheumatologist (arthritis specialist) or dermatologist (skin specialist) based on a combination of factors:

  • Presence of symptoms and physical signs
  • Characteristic skin changes (including location, size, shape, color)
  • Results of a complete medical history and physical exam

Other tests may be used to confirm the diagnosis or to determine the severity of the disease, such as:

  • Skin biopsy of affected skin, in which a small tissue sample is removed for evaluation under a microscope
  • Blood tests in which autoimmune proteins and kidney function are measured in a laboratory

Additional tests for systemic sclerosis

  • X-rays which can show changes in the skin, bones and internal organs such as the lungs and intestines
  • Tests to evaluate the swallowing function of the esophagus (tube leading from the mouth to the stomach)
  • Pulmonary function test (breathing test) to make sure the lungs are working properly
  • Echocardiography (ultrasound of the heart) to look at the heart function

No single test or X-ray will prove the diagnosis of scleroderma, and not all children will need all these tests.

How is pediatric scleroderma treated?

There is no cure for scleroderma, but with proper diagnosis, it can be treated and controlled.

Team-centered approach

Treatment is specialized for each child, based on his or her overall health, medical history, type of scleroderma, severity, and presence of other symptoms.

For systemic sclerosis, a team of specialists, including various physicians (dependent on involvement of internal organs), nurses, physical and occupational therapists, psychologists and other health care professionals, provide comprehensive care for your child in a compassionate setting.

The team focuses on medically controlling the disease while decreasing the effects of scleroderma on your child's physical growth and emotional development. The location of the skin rash, and how deep it is, will impact how the child feels the skin rash will affect his or her appearance (cosmetic impact).

Scleroderma management may include family counseling regarding cosmetic, financial, emotional, social or disability issues.

Available treatments

Treatments include medications, skin protection, therapy and rarely, surgery.

Medication

The goal of medication is to control disease activity. Several different medications to treat the effects of scleroderma are currently being investigated.

Steroids, such as cortisone or prednisone, may be used to decrease inflammation in muscles, joints or rarely in the skin itself. Steroids can also be helpful in treating fatigue, or occasionally to treat the early stages of internal organ inflammation. Generally, steroids do not work in the later stages of systemic sclerosis.

Methotrexate is often used to treat scleroderma, especially the linear type of scleroderma.

Non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen and naproxen are sometimes used for children who have arthritis to decrease joint inflammation.

Medications that dilate blood vessels and improve blood flow are often used to treat Raynaud's phenomena.

Skin protection

Protecting the skin will help maximize blood flow to the skin, hands and feet, especially for children who have Raynaud's phenomenon. Here are some tips:

  • Avoid injury to the affected areas, especially the tips of the fingers and the toes.
  • Protect the child's hands and feet from cold. Keep rooms at a warm temperature and have the child wear an extra layer of clothing in the winter, as well as a hat (ear muffs), gloves and warm socks. Wool is warmer than cotton or synthetic fabrics, and several layers of thin clothing are better than one heavy or thick layer of clothing.
  • Avoid smoking or exposure to smoke.
  • Avoid cold medications that include pseudoephedrine.
  • Protect the child from excess sun exposure.
  • Avoid using astringents, body or facial scrubs or harsh detergents on the skin.
  • Use lotions as prescribed by your doctor to keep your child's skin soft.
Physical therapy

Basic stretching and guided exercise programs with physical and occupational therapists help your child maintain flexibility, joint range of motion, muscle strength and blood flow to the affected areas. Therapy will also help prevent joint contractures (or bends at the joints). Splints may be recommended if necessary.

Surgery

In rare instances orthopedic hand or cosmetic surgery may become necessary to correct severe joint contractures or skin deformities or scars. Before surgery can be performed, the disease must usually be in remission (a time when the disease is not active) for several years.

What is the outlook?

Scleroderma is a chronic and slowly progressive disease, lasting for months or years. The outlook depends on the type of scleroderma, where and how much skin is involved and whether or not internal organs are affected.

Localized scleroderma frequently progresses in the early stages, but often does not further change after the first 3 to 4 years. It usually resolves by adulthood. In systemic sclerosis, children who have lung, heart or kidney problems have the greatest risk for complications. In linear, morphea and systemic sclerosis, joint contractures, cosmetic changes and uneven bone growth may occur.

Although scleroderma often does not go away, it can remain at the same level without getting worse for several years.

Through current research, we hope to develop a better understanding of the cause and improve treatment options.

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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 1/2/2009...#4910