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Parry Romberg syndrome with localized scleroderma: A case report
Mohsin Khan1, Mubeen Khan2, Raju Negi3
1M.D.S, Masters Of Dental surgery. Department of Orthodontics and Dentofacial Orthopaedics, Bangalore, Karnataka, India.
Journal of Clinical and Experimental Dentistry
|August 20, 2014
Summary
Parry Romberg syndrome (PRS) is a rare neurocutaneous disorder causing progressive hemifacial atrophy. This case highlights PRS associated with localized scleroderma, emphasizing its complex presentation and potential comorbidities.
Area of Science:
- Neurocutaneous disorders
- Dermatology
- Neurology
Background:
- Parry Romberg syndrome (PRS) is a rare, acquired neurocutaneous disorder of unknown etiology.
- Characterized by slow, progressive atrophy affecting one half of the face (hemifacial atrophy).
- Etiology is unclear, with theories suggesting cerebral fat metabolism disturbance and links to "en coup de sabre" morphea.
Observation:
- Presents a rare case of a 22-year-old female with PRS.
- The patient exhibited PRS associated with localized scleroderma.
- This case underscores the potential for associated neurological, cutaneous, ocular, dental, and autoimmune abnormalities.
Findings:
- Review includes classical clinical, radiographic, and histological findings of PRS.
- Discusses the ill-defined relationship between PRS and "en coup de sabre" morphea.
- Highlights the invalidating nature of PRS and its diverse associated conditions.
Implications:
- Emphasizes the need for comprehensive evaluation in PRS cases due to associated comorbidities.
- Suggests further research into the pathogenesis of PRS and its link to fat metabolism.
- Informs clinical management strategies for progressive hemifacial atrophy and associated localized scleroderma.
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