UMMS Affiliation
Department of Dermatology; Department of Anatomic Pathology; School of Medicine
Publication Date
2019-02-12
Document Type
Article
Disciplines
Nervous System Diseases | Skin and Connective Tissue Diseases | Stomatognathic Diseases
Abstract
Parry-Romberg Syndrome (PRS), or progressive hemifacial atrophy, is an uncommon disorder characterized by progressive unilateral loss of adipose tissue and underlying structures including muscle, cartilage, and bone, often with little or no sclerosis. PRS and morphea en coup de sabre (ECDS) have significant overlap, often coexist, and are likely different phenotypes of morphea.1 PRS usually presents in the first decade of life, but later presentations have been described.1 It is more common in females and the pathogenesis is not completely understood.1 Neurologic symptoms are the most common extracutaneous systemic manifestation. Bilateral disease occurs in rare instances.1 We describe a woman with profound bilateral facial atrophy whose presentation does not follow the typically reported disease course or histopathologic findings seen in PRS.
Keywords
ECDS, en coup de sabre, MRI, magnetic resonance imaging, PRS, Parry-Romberg syndrome, en coup de sabre, lipodystrophy, morphea
Rights and Permissions
Copyright © 2018 by the American Academy of Dermatology, Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
DOI of Published Version
10.1016/j.jdcr.2018.11.015
Source
JAAD Case Rep. 2019 Feb 12;5(3):209-212. doi: 10.1016/j.jdcr.2018.11.015. eCollection 2019 Mar. Link to article on publisher's site
Journal/Book/Conference Title
JAAD case reports
Related Resources
PubMed ID
30809561
Repository Citation
Tkachenko E, Cunningham MJ, O'Donnell P, Levin NA. (2019). Adult-onset bilateral Parry-Romberg syndrome. Open Access Articles. https://doi.org/10.1016/j.jdcr.2018.11.015. Retrieved from https://escholarship.umassmed.edu/oapubs/3778
Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License.
Included in
Nervous System Diseases Commons, Skin and Connective Tissue Diseases Commons, Stomatognathic Diseases Commons
Comments
Co-author Elizabeth Tkachenko is a medical student at UMass Medical School.