J Korean Cleft Palate-Craniofac Assoc.  2005 Oct;6(2):133-135.

A Case of Severe Scleroderma en Coup de Sabre with Ipsilateral Body Atrophy

Affiliations
  • 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.

Abstract

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.

Keyword

Scleroderma; Hemifacial atrophy; Total hemiatrophy; Two-jaw surgery; Groin free flap

MeSH Terms

Atrophy*
Cartilage
Congenital Abnormalities
Extremities
Facial Hemiatrophy
Free Tissue Flaps
Groin
Head
Humans
Scalp
Scleroderma, Localized
Skin
Strikes, Employee
Subcutaneous Tissue
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