Linear scleroderma is a localized scleroderma sub-type that is usually characterized by a distinct line or band of thickened skin that may limit the motion of bones and muscles underneath it.

It appears mostly during childhood on just one side of the body in the arms, legs, or forehead but possibly in more than one area. In addition to lesions on the face or scalp, there may also be abnormalities in muscles and fat tissue. When the face is affected, it may appear as an indentation on the forehead with the skin smooth, shiny, and hard. The condition is termed “en coup de sabre” because it may seem like a scar from a sword wound.

Linear scleroderma is the most common form of childhood scleroderma. Both sexes are affected equally. Sometimes it can result in one arm or leg growing faster than the other. Most linear scleroderma cases do not include Raynaud’s phenomenon.

Diagnosis is usually based on visible changes in the skin; blood tests that may show a positive antinuclear antibody; skin biopsy; diagnostic scans such as echocardiogram, electrocardiogram, or X-ray; and other tests that may show bone, soft tissue, and heart muscle abnormalites.

There is no current cure for linear scleroderma but treatments exist to ease symptoms. Physical therapy may be helpful for muscle strength, surgery could reduce fat or remove abnormal tissue, and medications can reduce inflammation and pain.

Symptoms of linear scleroderma may resemble other medical conditions, such as Parry-Romberg syndrome. Doctors recommend diagnosis confirmation before starting treatment.

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