Localized scleroderma is an inflammatory condition that causes hard and thickened patches of skin to form on different areas of the body. Unlike systemic scleroderma, localized scleroderma usually does not affect internal organs.
Risk factors for localized scleroderma
Up to three in every 100,000 people are diagnosed with localized scleroderma every year, and the disease is more common among Caucasians and in women compared to men.
Certain gene variants have been linked to an increased risk of developing localized scleroderma. As many as 40 percent of people with a more severe disease may personally have, or have a family member who has, a rheumatological disease like rheumatoid arthritis or another autoimmune disease such as vitiligo.
Localized scleroderma can also be triggered by infections, skin trauma, radiation, or exposure to other environmental factors.
What happens in localized scleroderma?
Localized scleroderma is characterized by the accumulation of collagen in the skin, which lead to the appearance of thick, hard patches. Scientists are not exactly sure why this happens, although it is agreed that inflammation plays an important role. Some sources describe localized scleroderma as an autoimmune condition.
Types of localized scleroderma
Depending on the shape and area of skin affected, localized scleroderma can be divided into two groups: morphea and linear scleroderma.
Symptoms of localized scleroderma
Morphea is characterized by thick, hard patches of skin that are white or yellow, and ringed with a purplish halo that may itch but is usually not painful.
In linear scleroderma, there are thickened streaks of skin rather than patches, which more commonly extend deep into the skin. This type of localized scleroderma may affect growth in children. Linear scleroderma is further divided into two subgroups: linear scleroderma ‘en coup de sabre’ and Parry-Romberg syndrome.
Thickened skin may potentially develop across joints in all types of localized scleroderma, potentially interfering with movement.
Symptoms of localized scleroderma tend to come and go with periods where the disease flares up, and periods where symptoms remain relatively stable.
Diagnosing localized scleroderma
Localized scleroderma is usually diagnosed with a thorough examination of the skin. To confirm the diagnosis, a skin biopsy may be performed. Ultrasound or magnetic resonance imaging (MRI) may be used to assess the depth of the disease.
Blood tests to look for certain autoantibodies (antibodies that mistakenly attack healthy tissues in the body) may also be performed to help diagnose localized scleroderma.
Treatment and prognosis
Localized scleroderma is not a life-threatening condition, although skin changes may significantly affect patients’ quality of life. Managing localized scleroderma can be challenging as it is difficult to predict which patients may develop a severe disease that then requires a more aggressive treatment.
Mild cases may be treated with creams and ointments. If the disease affects deeper tissues, phototherapy, steroids, or medications that suppress the immune system may be needed.
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