Localized scleroderma is a condition characterized by varying degrees of hardening of the skin, which happens because of abnormal collagen deposits.
Unlike systemic scleroderma, localized scleroderma rarely involves internal organs. But it can affect tissue directly under the skin — such as the muscles, nerves, and bones. Localized scleroderma is more common in children, who have a condition known as juvenile scleroderma.
Morphea and its progression
Morphea is a term that doctor use to describe the skin changes in localized scleroderma, although it is sometimes used to describe the disease itself.
Morphea usually changes in a predictable fashion over time. The first sign of it is a purplish or reddish area of hardened, slightly depressed skin. The area then becomes bigger, developing a whitish center. The skin thins, tightens and becomes surrounded by a ring of darkened skin. Morphea may itch, but is usually painless.
Types of localized scleroderma
The type of localized scleroderma that a person has is based on the area of the body that’s affected, and the shape of the abnormality. The types are:
- Plaque morphea
- Bullous morphea
- Deep morphea
- Generalized morphea
- Linear scleroderma
Although patients have one predominant type of localized scleroderma, some may have a combination of morphea patterns.
Plaque morphea is the most common form of localized scleroderma in adults. Patients typically develop oval patches two to 20 centimeters in diameter at one or two sites and do not have other symptoms.
A person with generalized morphea has patches in more than two parts of the body.
This type of morphea is more common in women. Symptoms can be triggered by exercise and progress quickly over a few months. The patches tend to be thicker and may affect muscles and deeper tissue. Movement can be restricted if the skin over the joints is affected.
Patients with deep morphea — also known as subcutaneous morphea or morphea profunda — usually have only one patch of affected skin.
But because there is damage to underlying tissue, they are at increased risk of experiencing restricted movements.
In addition, ulcers, or sores, are more likely to form over these types of patches because the skin is more fragile, less elastic, and receives less blood.
Linear scleroderma is the most common form of localized scleroderma in children. Instead of patches, there are long, narrow streaks of hardened skin.
This type of scleroderma is the most likely to affect the muscles and bones beneath the skin, disturbing growth and causing bone deformities. Patients can lose hair if their scalp is affected.
Some patients have gradual shrinking of the skin on one side of the face, and both the fat tissue and bones under the skin. This type of linear scleroderma is called Parry-Romberg syndrome, or progressive hemifacial atrophy. The tongue muscles on the side of the face that’s affected can be underdeveloped, and patients can have dental problems due to altered teeth and jaw positions. Seizures, severe face pain, and eye problems can also occur.
Patients are said to have en coup de sabre scleroderma when they have a streak of hard thickened skin that extends from their forehead to their scalp. The phrase means “like a sword cut” in English.
The jaw, teeth, and tongue can also be affected in this condition, but unlike Parry-Romberg syndrome, the brain, eyes, and nerves in the face are usually spared.
Patients with linear scleroderma that affects the rest of the body can have features of both Parry-Romberg syndrome and en coup de sabre scleroderma.
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