Cargando…

Lower limb onset Parry–Romberg syndrome: an unusual presentation of a rare disease

Parry–Romberg syndrome (PRS) is characterized by progressive degeneration and atrophy of the cutaneous, subcutaneous connective tissues, muscles and bones. Classically, PRS is restricted to unilateral face but in 20% of patients may extend to other parts of the body including ipsilateral or contrala...

Descripción completa

Detalles Bibliográficos
Autores principales: Jain, Rajendra Singh, Kumar, Sunil, Srivastava, Trilochan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5782432/
https://www.ncbi.nlm.nih.gov/pubmed/29497540
http://dx.doi.org/10.1093/omcr/omw031
_version_ 1783295192084774912
author Jain, Rajendra Singh
Kumar, Sunil
Srivastava, Trilochan
author_facet Jain, Rajendra Singh
Kumar, Sunil
Srivastava, Trilochan
author_sort Jain, Rajendra Singh
collection PubMed
description Parry–Romberg syndrome (PRS) is characterized by progressive degeneration and atrophy of the cutaneous, subcutaneous connective tissues, muscles and bones. Classically, PRS is restricted to unilateral face but in 20% of patients may extend to other parts of the body including ipsilateral or contralateral arms, trunk and legs. We report a case of 24-year-old male who presented with insidious onset, gradually progressive deformity and muscle wasting of right lower limb followed by right side of face and chest for 8 years. The right side of the face showed hemiatrophy, coup de sabre and deviation of nose and chin toward the same side. The magnetic resonance imaging showed atrophy of right lower limb. Computed tomography with 3D facial reconstruction revealed atrophy of facial bones on right side. He was managed with physiotherapy and symptomatic treatment and planned for facial and ankle reconstructive surgery on follow-up.
format Online
Article
Text
id pubmed-5782432
institution National Center for Biotechnology Information
language English
publishDate 2016
publisher Oxford University Press
record_format MEDLINE/PubMed
spelling pubmed-57824322018-03-01 Lower limb onset Parry–Romberg syndrome: an unusual presentation of a rare disease Jain, Rajendra Singh Kumar, Sunil Srivastava, Trilochan Oxf Med Case Reports Case Report Parry–Romberg syndrome (PRS) is characterized by progressive degeneration and atrophy of the cutaneous, subcutaneous connective tissues, muscles and bones. Classically, PRS is restricted to unilateral face but in 20% of patients may extend to other parts of the body including ipsilateral or contralateral arms, trunk and legs. We report a case of 24-year-old male who presented with insidious onset, gradually progressive deformity and muscle wasting of right lower limb followed by right side of face and chest for 8 years. The right side of the face showed hemiatrophy, coup de sabre and deviation of nose and chin toward the same side. The magnetic resonance imaging showed atrophy of right lower limb. Computed tomography with 3D facial reconstruction revealed atrophy of facial bones on right side. He was managed with physiotherapy and symptomatic treatment and planned for facial and ankle reconstructive surgery on follow-up. Oxford University Press 2016-08-29 /pmc/articles/PMC5782432/ /pubmed/29497540 http://dx.doi.org/10.1093/omcr/omw031 Text en © The Author 2016. Published by Oxford University Press. 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 non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Case Report
Jain, Rajendra Singh
Kumar, Sunil
Srivastava, Trilochan
Lower limb onset Parry–Romberg syndrome: an unusual presentation of a rare disease
title Lower limb onset Parry–Romberg syndrome: an unusual presentation of a rare disease
title_full Lower limb onset Parry–Romberg syndrome: an unusual presentation of a rare disease
title_fullStr Lower limb onset Parry–Romberg syndrome: an unusual presentation of a rare disease
title_full_unstemmed Lower limb onset Parry–Romberg syndrome: an unusual presentation of a rare disease
title_short Lower limb onset Parry–Romberg syndrome: an unusual presentation of a rare disease
title_sort lower limb onset parry–romberg syndrome: an unusual presentation of a rare disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5782432/
https://www.ncbi.nlm.nih.gov/pubmed/29497540
http://dx.doi.org/10.1093/omcr/omw031
work_keys_str_mv AT jainrajendrasingh lowerlimbonsetparryrombergsyndromeanunusualpresentationofararedisease
AT kumarsunil lowerlimbonsetparryrombergsyndromeanunusualpresentationofararedisease
AT srivastavatrilochan lowerlimbonsetparryrombergsyndromeanunusualpresentationofararedisease