What is juvenile localized scleroderma?

Scleroderma is a condition in which the skin becomes unusually thick and hard. The word scleroderma comes from two Greek words: “sclera,” meaning hard, and “derma,” meaning skin.

Scleroderma is an autoimmune disease in which the person’s immune system causes inflammation in the skin. The inflammation can trigger connective tissue cells to produce too much collagen, a fibrous protein that is a major part of many tissues. Excess collagen can lead to fibrosis, which is like scarring.

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. Another name for localized scleroderma is morphea.

What causes juvenile localized scleroderma?

The exact cause of juvenile localized scleroderma has not been found. Patients who have localized scleroderma are born with genetic (inherited) factors to have an overactive immune system. However, not all patients with these genetic predisposition develop the disease. In some patients, some environmental trigger kicks off a response in the immune system that causes localized scleroderma. These possible triggers include infection, trauma or certain drugs.

What are the symptoms of juvenile localized scleroderma?

Juvenile localized scleroderma usually begins as red-to-purplish patches with normal skin texture and thickness. Over time, the patches become hard and swollen, with a whitish or yellowish waxy center surrounded by a pinkish or purplish halo. In late stages, the affected areas become brown and then white.

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

  • 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.
  • 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.
  • 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 because of 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 (including muscle and bone).

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