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Surgical Management of Localized Scleroderma

BACKGROUND: Localized scleroderma is characterized by a thickening of the skin from excessive collagen deposits. It is not a fatal disease, but quality of life can be adversely affected due to changes in skin appearance, joint contractures, and, rarely, serious deformities of the face and extremitie...

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Autores principales: Lee, Jae Hyun, Lim, Soo Yeon, Lee, Jang Hyun, Ahn, Hee Chang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Cleft Palate-Craniofacial Association 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5647850/
https://www.ncbi.nlm.nih.gov/pubmed/29090197
http://dx.doi.org/10.7181/acfs.2017.18.3.166
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author Lee, Jae Hyun
Lim, Soo Yeon
Lee, Jang Hyun
Ahn, Hee Chang
author_facet Lee, Jae Hyun
Lim, Soo Yeon
Lee, Jang Hyun
Ahn, Hee Chang
author_sort Lee, Jae Hyun
collection PubMed
description BACKGROUND: Localized scleroderma is characterized by a thickening of the skin from excessive collagen deposits. It is not a fatal disease, but quality of life can be adversely affected due to changes in skin appearance, joint contractures, and, rarely, serious deformities of the face and extremities. We present six cases of localized scleroderma in face from our surgical practice. METHODS: We reviewed six localized scleroderma cases that were initially treated with medication and then received follow-up surgery between April 2003 and February 2015. Six patients had facial lesions. These cases presented with linear dermal sclerosis on the forehead, oval subcutaneous and dermal depression in the cheek. RESULTS: En coup de sabre (n=4), and oval-shaped lesion of the face (n=2) were successfully treated. Surgical methods included resection with or without Z-plasty (n=3), fat graft (n=1), dermofat graft (n=1), and adipofascial free flap (n=1). Deformities of the affected parts were surgically corrected without reoccurrence. CONCLUSION: We retrospectively reviewed six cases of localized scleroderma that were successfully treated with surgery. And we propose an algorithm for selecting the best surgical approach for individual localized scleroderma cases. Although our cases were limited in number and long-term follow-up will be necessary, we suggest that surgical management should be considered as an option for treating scleroderma patients.
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spelling pubmed-56478502017-10-31 Surgical Management of Localized Scleroderma Lee, Jae Hyun Lim, Soo Yeon Lee, Jang Hyun Ahn, Hee Chang Arch Craniofac Surg Original Article BACKGROUND: Localized scleroderma is characterized by a thickening of the skin from excessive collagen deposits. It is not a fatal disease, but quality of life can be adversely affected due to changes in skin appearance, joint contractures, and, rarely, serious deformities of the face and extremities. We present six cases of localized scleroderma in face from our surgical practice. METHODS: We reviewed six localized scleroderma cases that were initially treated with medication and then received follow-up surgery between April 2003 and February 2015. Six patients had facial lesions. These cases presented with linear dermal sclerosis on the forehead, oval subcutaneous and dermal depression in the cheek. RESULTS: En coup de sabre (n=4), and oval-shaped lesion of the face (n=2) were successfully treated. Surgical methods included resection with or without Z-plasty (n=3), fat graft (n=1), dermofat graft (n=1), and adipofascial free flap (n=1). Deformities of the affected parts were surgically corrected without reoccurrence. CONCLUSION: We retrospectively reviewed six cases of localized scleroderma that were successfully treated with surgery. And we propose an algorithm for selecting the best surgical approach for individual localized scleroderma cases. Although our cases were limited in number and long-term follow-up will be necessary, we suggest that surgical management should be considered as an option for treating scleroderma patients. The Korean Cleft Palate-Craniofacial Association 2017-09 2017-09-26 /pmc/articles/PMC5647850/ /pubmed/29090197 http://dx.doi.org/10.7181/acfs.2017.18.3.166 Text en Copyright © 2017 The Korean Cleft Palate-Craniofacial Association http://creativecommons.org/licenses/by-nc/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Article
Lee, Jae Hyun
Lim, Soo Yeon
Lee, Jang Hyun
Ahn, Hee Chang
Surgical Management of Localized Scleroderma
title Surgical Management of Localized Scleroderma
title_full Surgical Management of Localized Scleroderma
title_fullStr Surgical Management of Localized Scleroderma
title_full_unstemmed Surgical Management of Localized Scleroderma
title_short Surgical Management of Localized Scleroderma
title_sort surgical management of localized scleroderma
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5647850/
https://www.ncbi.nlm.nih.gov/pubmed/29090197
http://dx.doi.org/10.7181/acfs.2017.18.3.166
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