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What is LINEAR SCLERODERMA?

Scleroderma is a rare autoimmune disease in which an overproduction of abnormal collagen causes normal tissues to be replaced with thick, dense scar tissue that can affect underlying bones and muscles if left untreated.

Symptoms SYMPTOMS

Linear scleroderma is a progressive loss of subcutaneous fat with pigment changes in the skin. It is a type of localized scleroderma in which the area of skin affected appears in a band. It typically first appears in young children on one side of the body. It can affect the trunk, arms, legs, face or neck, or multiple parts of the body. Symptoms of localized scleroderma may include:

  • Shiny, thickened patches of skin
  • Discolored (lighter or darker) skin
  • Joint tightness

Scleroderma can result in cosmetic problems, scarring, growth abnormalities and limited motion if joints are affected. Symptoms may resemble other medical conditions, so always consult your child’s physician to confirm her diagnosis before pursuing treatment.

Diagnosis of linear scleroderma is usually based on the changes in the skin and internal organs. Because linear scleroderma is often associated with a positive antinuclear antibody, an antibody test may help distinguish the type of scleroderma present.

Treatment TREATMENT & MANAGEMENT

Treatment for scleroderma depends on your child’s overall health and the severity of the condition. Treatment may include:

Medication - Your child's medical team may recommend medications such as nonsteroidal, anti-inflammatory medications (NSAIDs) or corticosteroids to relieve pain; penicillamine to slow the thickening process and delay damage to internal organs; or immunosuppressive medications.

Surgery - Surgical treatment may involve fat transferred by injection, grafting of dermal fat grafts, or excision of isolated patches of abnormal tissue. This is a minimally invasive procedure and usually can be done on an outpatient basis.

If large areas of discolored, irregular skin are involved, the preferred treatment option may be direct excision of the abnormal tissue with closure of the adjacent normal skin. In severe atrophy, large amounts of tissue may be transferred to the affected area using microsurgical techniques. This transferred tissue often comes from the trunk or legs. The tissue may be placed deeply to add volume, closer to the surface of the skin to replace damaged skin, or both.