Progressive systemic sclerosis: rare localization of the maxillofacial district.

The authors focus on a case of scleroderma, or progressive systemic sclerosis. Scleroderma is a disease of unknown cause, characterized by an abnormal synthesis of connective tissues that causes a sclerosis of the skin involving organs and systems at various levels. It may appear in different forms and may involve, although not frequently, the maxillofacial district. The authors examined a 21-year-old woman who had been diagnosed with systemic progressive sclerosis in 1994. The patient showed a strong limitation of oral aperture, evidenced by an electrognathographic test, and osseous changes. Panoramic and full-mouth intraoral radiographs showed a complete agenesis of coronoid processes on both sides, whereas the angles and the rising branches of the mandibular and periodontal ligaments appeared normal. The patient was diagnosed as having maxillofacial localization of scleroderma with involvement of coronoid processes bilaterally.