Parry-Romberg syndrome with Linear Scleroderma
- VernacularTitle:Парри-Ромбергийн хам шинжийн шугаман склеродермтэй хавсарсан тохиолдол
- Author:
Otgonbayar J
1
;
Tuvshinjargal S
1
;
Natsagdorj L
1
Author Information
1. Third State Central Hospital
- Publication Type:Case Reports
- Keywords:
Progressive hemifacial atrophy;
Neurocutaneous disorder;
Electroneuromyography
- From:
Mongolian Journal of Health Sciences
2026;96(6):250-254
- CountryMongolia
- Language:Mongolian
-
Abstract:
Background:Parry-Romberg syndrome (PRS) is a rare acquired neurocutaneous disorder characterised by progressive hemifacial atrophy involving subcutaneous tissue, muscle, and occasionally bone. Linear scleroderma (also known as Mophea en coup de sabre) is a subtype of focal scleroderma, characterised by thickening and hardening of the skin due to excessive collagen deposition in the skin and subcutaneous tissue. Both disorders occur together, although rarely. We present a case of Parry-Romberg Syndrome with Linear Scleroderma, which initially started on the left side of the face but gradually progressed over the years and, in recent years, has spread to the right side of the face and tongue, and is being treated with immunosuppressive therapy.
Case Presentation:A woman in her 30s presented with progressive soft-tissue atrophy of the left side of the face, extending from the forehead and parietal region to the cheek and mandible, with associated left frontal pain and facial asymmetry. The condition had begun at approximately 10 years of age with localized alopecia and gradually progressed over several years, later involving the left side of the tongue. Examination revealed marked left-sided facial soft-tissue atrophy, tongue muscle atrophy, enophthalmos, and facial asymmetry. Electroneuromyography demonstrated chronic myogenic changes in muscles innervated by the left trigeminal, facial, and hypoglossal nerves. Brain magnetic resonance imaging showed thinning of the skull and subcutaneous soft tissues in the left frontoparietal region, with associated reduction in the size of the left orbital soft tissues and globe. Based on the clinical and imaging findings, Parry–Romberg syndrome associated with linear scleroderma was diagnosed, and immunosuppressive therapy was initiated with longterm follow-up.
Conclusion:Parry-Romberg syndrome is a rare, slowly progressive disorder that can involve the skin, subcutaneous tissue, muscles, bone, eyes, and nervous system. In patients presenting with progressive hemifacial atrophy, detailed neurological examination and appropriate imaging studies are important for establishing the diagnosis and identifying associated neurological involvement. This case highlights the clinical importance of recognizing Parry–Romberg syndrome and its association with linear scleroderma.
- Full text:202609270105212978144_Парри-Ромбергийн хам шинжийн шугаман склеродермтэй хавсарсан тохиолдол.pdf
