Written by: Dr. Ronald Laxer Pediatric Rheumatologist The Hospital For Sick Children Toronto, Canada Dr. James R. Seibold Rheumotologist Rheumatology - Scleroderma Program New Brunswick, New Jersey Vanessa Malcarne, Ph.D Psychology Annex for Research and Training San Diego, California Kathy Gaither Parent & JSDN - Founder/President

What is Juvenile Scleroderma?

Scleroderma is a connective tissue disease involving skin, blood vessels and the immune system. In its systemic form, internal organ involvement can occur.

Scleroderma means "hard skin". Thickening and tightening of the skin occurs from a buildup of collagen* and other natural skin proteins which in turn is thought to be driven by a local inflammatory/immune response.
(*Collagen - a fibrous protein made by cells that provides the firmness in the skin, forms the lining of organs, and is the basic structural protein in bones, tendons, ligaments, and joints).

There are a variety of distinct and separate syndromes that result in scleroderma.

1. Localized - not involving internal body systems. Localized is much more common in children than adults.

1. Morphea: patches of thickened, waxy, ivory, or yellow - white shiny lesions.

2. Generalized Morphea: the lesions are a combination of morphea and linear, and involve almost the entire skin.

3. Linear: thickened skin is in the pattern of a line down an arm or leg. Pigment changes both dark skin and patchy areas of lighter skin. It can interfere with the growth of the limb due to atropy*.
   (*atropy - muscles that do not move will weaken and shrink).

4. En Coupe de Sabre: indentation on the forehead or at the frontal hairline. A thickened skin can spread to the entire face. Atrophy of the lower part of the face is called Parry-Romberg Syndrome.

5. Eosinophilic Fasciitis (EF): A sclerodermalike disorder, white blood cells attack the fascia, which is a thin sheet of tissue that seperates muscle from the fat and skin above it. In some cases the fascia becomes inflamed. EF affects the arms, legs, and trunk while sparing the hands and face.
   
   Localized DOES NOT OVERLAP with Systemic Scleroderma.

2. Systemic - involving internal systems. Systemic is much less frequent in children.

1. Limited: late internal organ involvement if any. Also known as
   "CREST".
   Calcinosis - calcium deposits in the skin
   Raynaud's - fingers and toes turn white, blue, red when exposed to cold temperature or stress.
   Esophageal dysmotility - frequent heartburn, difficulty swallowing
   Sclerodactyly - thickening of the skin of the fingers causing contractures* in the fingers in a bent flexed position
   (*contracture - bending of a joint, the inability to fully straighten out a joint).
   Telangiectasias- areas of red, prominent blood vessels in the skin

2. Diffuse: generalized skin involvement and early internal organ involvement, especially lungs and gastrointestinal system (esophageal dysmotility, poor food absorption, constipation). Raynaud's is very common. Involvement of heart, kidney, muscles and joints also occurs.

How Many Children Suffer from Juvenile Scleroderma?

This rare disease affects approximately 5,000 to 7,000 children. Since JSD is  commonly undiagnosed and often misdiagnosed, the number of children affected may be even greater.

What is The Treatment for Juvenile Scleroderma?

The cause is unknown. No cure has been discovered. Treatment of JSD remains a dilemma, especially in regards to which children should be treated and how the treatment should be monitored. However, there are a number of treatments available. The most common medications used to treat symptoms of JSD are chemotherapy, corticosteroids, and immunosuppressants. Any of these treatments can cause severe side effects.

Emotional Effects of Juvenile Scleroderma

Children who suffer from JSD may experience a variety of emotions. They may be confused by their illness, and angry they are sick, especially if their illness puts limits on their activities. When the future is uncertain they may become frustrated, worried, and/or depressed. The often visible changes in appearance may be particularly difficult for children to handle, since they may lead to teasing by other children and are a daily reminder of the illness. Parents need to talk openly with their children about JSD, and keep a watchful eye for signs of distress. Also, parents and children can become involved in a support network to exchange information and find emotional reassurance.

How Does the Juvenile Scleroderma Network Help?

It is our mission to help families who have children affected by JSD. Because these families are often battling it out on their own without support, it is important they know support is only a phone call away.

For a printable version of this document, click here.

For more information on Juvenile Scleroderma, contact:

Juvenile Scleroderma Network, Inc.
1204 W. 13th Street, San Pedro, CA 90731

Tel: (310)519-9511 (Pacific Time)
24 Hour Support Line: 1-866-338-5892 (toll-free)
Speak to another JSD parent for emotional and logistical support provided by home-based JSD volunteers. For medical advice, please contact your child's physician.

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