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Journal of the Korean Cleft Palate-Craniofacial Association 2005;6(2):133-135.
A Case of Severe Scleroderma en Coup de Sabre with Ipsilateral Body Atrophy.
So Min Hwang, Chul Sun Kang, David J David
1Aesthetic, Plastic and Reconstructive Surgery Center, Good Moonhwa Hospital, Busan, Korea. smhwang@moon hwa.or.kr
2Australian Craniofacial Unit, Women's & Children's Hospital, Adelaide, Australia.
Linear scleroderma is a variant of localized scleroderma which may occur in the head, trunk and extremities, affecting variously the skin, subcutaneous tissue, muscle, cartilage and bone. Particularly, scleroderma en coup de sabre is the descriptive term denoting linear scleroderma of the frontoparietal area of the scalp and face. We report a case of severe scleroderma en coup de sabre with ipsilateral body atrophy(total hemiatrophy) who underwent multidisciplinary assessment and required both two-jaw surgery and groin free flap to correct the underlying skeletal deformities and augment the facial soft tissues. The reason for classifying this as a case of scleroderma en coup de sabre in preference to Parry-Romberg syndrome is because of the striking clinical features seen in this patient.
Keywords: Scleroderma; Hemifacial atrophy; Total hemiatrophy; Two-jaw surgery; Groin free flap
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