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Diseases & Conditions

Juvenile Localized Scleroderma

What is juvenile localized scleroderma?

Scleroderma is a condition in which the skin becomes unusually thick and hard.

Juvenile localized scleroderma refers to disease that affects children. The term "localized" means that it mainly involves the skin, connective tissue, muscle, and bone (unlike systemic sclerosis, which can affect organs deep inside the body). Females are slightly more likely to have the disease than males.

What are the symptoms of juvenile localized scleroderma?

Juvenile localized scleroderma usually begins as patches of yellowish or ivory-colored stiff, dry skin. The patches become hard, slightly depressed, oval-shaped plaques with a whitish or yellowish center surrounded by a pinkish or purplish halo.

There are five different types of localized scleroderma, each with its own symptoms:

  • Linear morphea – This is the most common type of morphea in children. Long plaques appear in lines across the body or traveling in the same direction as the arms and legs. The thickened skin can affect underlying bone and muscle and limit the motion of the joints and muscles. This can cause limb defects, poor growth, and disabilities. If the face or scalp are affected, the condition is known as "en coup de sabre" (because it looks as if the person has been struck by a sword). En coup de sabre appears as an indented, vertical, colorless line of skin on the forehead.
  • Circumscribed or plaque morphea – This is the least harmful of the morphea types. It mainly affects the skin, and occasionally the tissue just under the skin. The plaques are small, few in number, and appear on only one or two areas of the body.
  • Generalized morphea – In this condition, there are four or more plaques affecting two or more areas of the body (usually the trunk and legs). Individual plaques may spread and join one another.
  • Bullous morphea – When the skin blisters or bubbles, it is said to be bullous. This condition can occur due to some type of trauma at the site of the plaque, or because the normal flow of lymphatic fluid is blocked.
  • Deep morphea – This is the most harmful form, but is very rare. It usually occurs in the tissue just under the skin.

What causes juvenile localized scleroderma?

The exact cause of juvenile localized scleroderma has not been found, but researchers are looking into a number of possibilities:

  • Overproduction of collagen, the material that helps make up connective tissue
  • Autoimmune disorders, in which the body’s immune system turns against itself. Patients with localized scleroderma often have such autoimmune conditions as Hashimoto’s thyroiditis, vitiligo (loss of skin pigmentation), and type 1 diabetes.
  • Certain drugs or environmental toxins
  • Infections
  • Injury to the skin. Wound- healing proteins may play a role in scleroderma.

Juvenile localized scleroderma does not reduce a person's life expectancy.

How is juvenile localized scleroderma diagnosed?

Diagnosis can be difficult because juvenile localized scleroderma is a relatively rare disease. Also, in the early stages of scleroderma, it may be easily mistaken for other systemic autoimmune diseases.

There are no specific tests available to confirm a diagnosis. The doctor may diagnose scleroderma in the following ways:

  • Examining the lesions
  • Taking a biopsy (sampling) of skin or underlying tissue
  • A test called infrared thermography to measure temperature changes on the skin. This can help determine abnormal areas of tissue growth.
  • A computerized skin score, which uses scanned images of skin lesions over time to measure their dimensions
  • Ultrasound to measure blood flow through the tissues and depth of lesions
  • Magnetic resonance imaging (MRI) to measure the depth of lesions

How is juvenile localized scleroderma treated?

Treatment of juvenile localized scleroderma is aimed at keeping the disease from getting worse. Treatment usually lasts 2-3 years and includes some combination of the following options:

  • Topical, oral, or intravenous steroids
  • Drugs that reduce the inflammation and skin changes, including methotrexate and mycophenolate
  • Physical therapy and massage to improve muscle strength and maintain joint mobility
  • Skin creams containing lanolin to help relieve dryness and itching
  • Phototherapy using ultraviolet (UV) rays to help soften plaques. This must be done carefully, as it can prematurely age the skin or lead to skin cancer.
  • Vitamin D

What is the prognosis (outlook) for patients with juvenile localized scleroderma?

Juvenile localized scleroderma often affects only skin tissues and does not harm major organs. It may get better or go away on its own.

Recurrence of old lesions or development of new skin lesions occurs in less than 20% of patients five years after initial onset of scleroderma symptoms.

It is exceedingly rare to see localized scleroderma transform into the more serious systemic sclerosis.

Some patients may end up with hardened skin, while others may suffer damage to the skin or limbs. Surgery may be necessary if the disease was not well-controlled when the patient was younger, and if there are deformities in the bones or face. Surgery should not be done until the child has finished growing and the disease is inactive.

Can juvenile localized scleroderma be prevented?

There is no known way to prevent juvenile localized scleroderma. It is not contagious: you cannot get scleroderma by shaking hands, hugging, kissing, sexual contact, contact with blood or bodily fluids, sharing eating utensils, or by airborne contact from coughing or sneezing. Scleroderma is not passed down along family lines.

References

National Institute of Arthritis and Musculoskeletal and Skin Diseases. Scleroderma. www.niams.nih.gov. Accessed September 1, 2011.

Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology 2006;45:614–620. rheumatology.oxfordjournals.org. Accessed September 1, 2011.

American College of Rheumatology. Localized Scleroderma. www.rheumatology.org. Accessed September 1, 2011.

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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: 9/1/2011…#14857

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