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Linear Scleroderma

Linear Scleroderma


Linear scleroderma is an autoimmune condition that causes focal atrophy of the skin, fat and muscle of the brow, forehead and scalp. There is also variable increased or decreased pigmentation of the skin. The classic finding of linear scleroderma is called the en coup de sabre (Strike of the Sabre) – a vertical, linear indentation of the forehead. Hairless patches (alopecia) may also be found in the scalp and occasionally the upper eyelid and the bone of the eye socket (orbit) can be involved. Linear scleroderma falls under the same umbrella diagnosis of Morphea, also called localized scleroderma. Morphea is an autoimmune condition that results in atrophy of the subcutaneous tissue and changes in the skin quality and pigmentation in other parts of the body also. It is not known why these focal autoimmune conditions occur and why they selectively affect different parts of the body and face.

TREATMENT

How and when to treat patients with linear scleroderma remains controversial. The conventional way to approach treatment was to let the disease continue to progress until a final steady state was achieved. This was also called letting the disease “burn out.” Today a number of immune modulators may be used to slow or arrest the progression of the tissue loss. Some experts believe that early attempts at reconstruction may also cause arrest or decrease the rate of progression of the disease. Careful coordination with the other providers involved in caring for you is important, including your dermatologist and/or rheumatologist. The main way to treat the changes in the soft tissues of the forehead and brow is called fat grafting or fat transfer.

HOW IS THE SURGERY PERFORMED?

Fat grafting uses the patient’s own fat that is harvest by performing liposuction. Typically the fat is harvested from the flank, hip or abdomen. Usually only 1-2 tablespoons of fat can be injected per treatment because the tissues are very thin. The fat is injected between the skin and muscle and between the muscle and bone in the areas where thinning of the tissues has occurred. Special thin, blunt injection needles are used to avoid injury to the skin, muscles, nerves and blood vessels in the treated area. The treatment must be performed 1-3 times, depending on the severity of the soft tissue loss. Fat grafting is performed at 4-6 months intervals between each treatment to allow the tissues to completely heal between treatments.

RECOVERY

Typically patients require prescribed pain medicine for a day or two and then just over the counter pain medications as needed for a week. Mild soreness at the donor site and recipient sites is common. Patients return back to school, work and activities of daily living in 1 week. They resume normal exercise and sports in 2-3 weeks.

AM I A CANDIDATE FOR FAT GRAFTING

The best candidates for surgery are healthy and do not smoke. Fat grafting can be safely performed in almost everyone.

Common problems treated with fat grafting include:
  • Thinning of the temples
  • Linear depression of the forehead (en coup de sabre)
  • Depression of the forehead bone or brow (bone under eyebrow)

COST OF SURGERY

Fat grafting for the treatment of linear scleroderma is typically covered by insurance.

WHY CHOOSE DR. DERDERIAN?

During his plastic surgery residency at New York University Dr. Derderian had extensive exposure to the surgical treatment of patients with Linear Scleroderma and Parry Romberg syndrome. Dr. Derderian is board certified by the American Board of Plastic Surgery. He performs clinical research with Dr. Heidi Jacobi who is a dermatologist at UT Southwestern and a recognized expert in treating patients with Morphea, Parry Romberg and Linear Scleroderma. Their research includes evaluating the three dimensional changes in facial symmetry that occur with disease progression and after treatment with fat grafting. Dr. Derderian frequently performs fat grafting for patients with Linear Scleroderma and Parry Romberg syndrome at both UT Southwestern and Children’s Health in Dallas, TX.

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