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Scleroderma (sometimes referred to as morphea) is a rare autoimmune disorder that leads to scarring of the skin, joints, and sometimes internal organs. Scleroderma literally means ‘hard skin’ and is caused when normal skin cells are attacked by the immune system and replaced by thick, dense scar tissue.
There are two forms of scleroderma:
Scleroderma is caused by defects in a child’s immunity when the body breaks down and reacts adversely to its own tissues. Immune systems in scleroderma patients are thought to trigger other cells to produce excess collagen, which is secreted into the skin (and sometimes organs), leading to the hardening and scarring.
Sudden changes in the appearance and feeling of skin may indicate scleroderma. Common symptoms include:
Scleroderma is usually diagnosed by a pediatric rheumatologist in conjunction with a dermatologist. A diagnosis may result from a combination of the following:
X-rays and other scans may be needed to determine if scarring has spread to internal organs.
In children, localized scleroderma (affecting just the skin) can typically be treated with creams and ointments. Steroid medications may also be helpful in reducing inflammation in the muscles and joints so that the skin does not feel so tight. Physical therapy may also be prescribed to promote flexibility.
In rare cases, if scarring is detected on any organs, surgery may be necessary to remove the damaged tissue and restore proper organ function.