PROGRESSIVE HEMIFACIAL ATROPHY (PARRY-ROMBERG SYNDROME)

By Gary M. White, MD

Parry-Romberg Syndrome or Progressive Hemifacial Atrophy


Also known as Parry-Romberg syndrome, or Parry-Romberg facial hemiatrophy, this disease is characterized by atrophy of one side of the facial skin with additional variable involvement of the subcutaneous muscle, fat, cartilage, bone, and brain. The onset is typically in the first two decades of life. This disease may be similar to morphea and some patients have localized scleroderma on other parts of the body. It has been reported to be caused by Borrelia but this has not been proven. En coup de sabre is a related condition.

Clinical

The skin on one side of the face becomes atrophic and may take on a bluish hue. Slow progression is seen over time. There may be ocular complications if the lesion encroaches on the eye. There may be atrophy of the subcutaneous tissue, muscle, and bone with little or no skin involvement. This condition often results in severe facial asymmetry.

Treatment

A multidisciplinary approach involving the dermatologist, ophthalmologist, and neurologist is important. If caught early, phototherapy using UVA1 can be quite effective. Otherwise methotrexate along with prednisone are usually given. See morphea in a child. ANA and Borrelia titers may be checked. Reconstructive surgery involving fat transplantation, flaps, or other techniques may be beneficial once progression has stopped.

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