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

Comments

Co-author Elizabeth Tkachenko is a medical student at UMass Medical School.

Related Resources

Link to Article in PubMed

PubMed ID

30809561

Creative Commons License

Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License.

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