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 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.

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.