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Progressive systemic sclerosis: rare localization of the maxillofacial district
P Cascone1, A Rivaroli, S Vetrano
1Department of Maxillofacial Surgery, University of Rome La Sapienza, Italy.
The Journal of Craniofacial Surgery
|October 22, 1998
Summary
This study details a rare case of scleroderma affecting the maxillofacial region in a young woman. The progressive systemic sclerosis led to bilateral agenesis of the coronoid processes, significantly limiting oral aperture.
Area of Science:
- Rheumatology
- Dermatology
- Oral and Maxillofacial Surgery
Background:
- Scleroderma, or progressive systemic sclerosis, is a connective tissue disease of unknown etiology.
- It characteristically involves abnormal connective tissue synthesis, leading to sclerosis of the skin and potential organ system involvement.
- Maxillofacial manifestations of scleroderma, though infrequent, can significantly impact patient function.
Observation:
- A 21-year-old female patient diagnosed with systemic progressive sclerosis in 1994 presented with significant limitations in oral aperture.
- Diagnostic evaluation included electrognathography, confirming restricted jaw movement.
- Radiographic imaging revealed bilateral agenesis of the coronoid processes.
Findings:
- The patient exhibited bilateral complete agenesis of the coronoid processes.
- Other mandibular structures, including angles and rising branches, along with periodontal ligaments, appeared normal.
- The clinical presentation and radiographic findings confirmed maxillofacial localization of scleroderma.
Implications:
- This case highlights the importance of recognizing rare scleroderma manifestations in the maxillofacial region.
- Understanding these presentations is crucial for accurate diagnosis and management of functional limitations.
- Further research into the specific mechanisms of maxillofacial involvement in scleroderma may improve patient outcomes.