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KMID : 0032220110230030342
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Annals of Dermatology
2011 Volume.23 No. 3 p.342 ~ p.347
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Parry-Romberg Syndrome with En Coup de Sabre
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Jun Jae-Hun
Kim Ho-Youn
Jung Han-Jin
Lee Weon-Ju
Lee Seok-Jong
Kim Do-Won
Kim Moon-Bum
Kim Byung-Soo
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Abstract
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Parry-Romberg syndrome (PRS) is a relatively rare degenerative disorder that is poorly understood. PRS is characterized by slowly progressing atrophy affecting one side of the face, and is frequently associated with localized scleroderma, especially linear scleroderma, which is known as en coup de sabre. This is a report of the author’s experiences with PRS accompanying en coup de sabre, and a review of the ongoing considerable debate associated with these two entities. Case 1 was a 37-year-old woman who had right hemifacial atrophy with unilateral en coup de sabre for seven years. Fat grafting to her atrophic lip had been conducted, and steroid injection had been performed on the indurated plaque of the forehead. Case 2 was a 29-year-old woman who had suffered from right hemifacial atrophy and bilateral en coup de sabre for 18 years. Surgical corrections such as scapular osteocutaneous flap and mandible/maxilla distraction showed unsatisfying results.
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KEYWORD
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Encoup de sabre, Hemifacial atrophy, Parry-Romberg syndrome
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