Linear scleroderma is a type of localized scleroderma that is most commonly diagnosed in children.

Instead of patches of thickened skin commonly seen in the morphea-type of localized scleroderma, patients with the linear type have thick streaks of skin usually affecting the limbs and torso.

The condition is further divided into two subgroups: linear scleroderma “en coup de sabre” and Parry-Romberg syndrome.

Symptoms

Thick and hard bands of skin are the first symptoms to appear in linear scleroderma. These typically only affect one side of the body. They may also extend deeper into the skin, causing abnormalities in the fat tissue and muscles. If the skin hardens over a joint, movements may be limited and stiffened joints known as contractures may form.

In children, linear scleroderma may affect growth, and in severe cases, result in one arm or leg to grow faster than the other.

Symptoms may fluctuate, with periods of rapidly developing symptoms and times where symptoms remain relatively stable.

Patients with the “en coup de sabre” (sword wound or cut, in English) subtype usually have a one-sided thickening of skin across the face or scalp that may look like a sword cut. Soft tissues and muscles in the face, as well as the teeth, eyes, and brain, may also be affected. Patients may lose hair over areas of affected skin.

In Parry-Romberg syndrome (also known as progressive hemifacial atrophy), there may be a slow thinning or shrinking of the skin, muscles, bones, and soft tissues on one side of the face. Skin color may also change. In rare cases, both sides of the face may be affected.

The eyes, teeth, and brain may also be affected. If Parry-Romberg impacts the brain, it could cause seizures, migraines, or severe pain in the face known as trigeminal neuralgia.

Diagnosis

The diagnosis of linear scleroderma is usually based on visible changes in the skin and confirmed with a skin biopsy. Patients may also undergo a blood test to look for autoantibodies that can help confirm the diagnosis. X-rays and MRI scans (magnetic resonance imaging) may be performed to assess the extent of the disease.

Treatment

Linear scleroderma, like morphea, usually improves on its own without the need for treatment, although it usually lasts longer than morphea. However, it may cause long-term damage to the fingers, toes, and face, and any change in the size of the limbs may be permanent.  

Although there is currently no cure for linear scleroderma, there are several treatment options, such as medication, surgery, and physical therapy that can help relieve the condition’s symptoms.

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