title: Orofacial manifestations of scleroderma. A literature review.
abstract: BACKGROUND
  Scleroderma is a rare disease of the connective tissue (50 to 200 patients/1 million people; 60,000 patients in France). We conducted a literature review about the orofacial manifestations of scleroderma that have been little studied.
  
  
  METHODS
  The 45 articles found in 6 different databases by using the keywords "scleroderma", "systemic sclerosis", "oral medicine", "face" and published between 1944 and 2016 were selected, for a total of 328 patients.
  
  
  RESULTS
  A total of 1187 orofacial manifestations of scleroderma were identified, occurring mainly in women (84.5%) with a mean age of 40.2 years, 10 years on average after the first manifestation of the disease. The main ones were limitation of mouth opening (69.8%), widening of the periodontal ligament (67.3%), xerostomia (63.4%), telangiectasia (36.2%) and bone lesions (34.5%). Dental root resorptions, pulp and nose calcifications were also reported but with no evident link with scleroderma.
  
  
  CONCLUSIONS
  Orofacial manifestations of scleroderma are probably more common than reported. They mostly affect women with a mean age of 40. The most common oral manifestations are limitation of mouth opening, widening of the periodontal ligament and xerostomia. Because of the handicap they may be responsible for, these manifestations must be detected early in order to prevent from functional impairments and from dental and periodontal lesions.
authors.affiliation: Service de chirurgie maxillo-faciale, Hôpital de la Timone, 264, rue Saint-Pierre, 13385 Marseille, France; Aix-Marseille Université, Jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France; Aix Marseille Université, CNRS, LPL UMR 7309, 13100 Aix-en-Provence, France. Electronic address: mehdi.hadj-said@ap-hm.fr.
authors.forename: M
authors.initials: M
authors.lastname: Hadj Said
authors.affiliation: Service de chirurgie maxillo-faciale, Hôpital Nord, chemin des Bourrely, 13015 Marseille, France; Aix-Marseille Université, Jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France.
authors.forename: J M
authors.initials: JM
authors.lastname: Foletti
authors.affiliation: Service de chirurgie maxillo-faciale, Hôpital de la Timone, 264, rue Saint-Pierre, 13385 Marseille, France; Aix-Marseille Université, Jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France.
authors.forename: N
authors.initials: N
authors.lastname: Graillon
authors.affiliation: Service de chirurgie maxillo-faciale, Hôpital Nord, chemin des Bourrely, 13015 Marseille, France; Aix-Marseille Université, Jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France.
authors.forename: L
authors.initials: L
authors.lastname: Guyot
authors.affiliation: Service de chirurgie maxillo-faciale, Hôpital de la Timone, 264, rue Saint-Pierre, 13385 Marseille, France; Aix-Marseille Université, Jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France; Aix Marseille Université, CNRS, LPL UMR 7309, 13100 Aix-en-Provence, France.
authors.forename: C
authors.initials: C
authors.lastname: Chossegros
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005145:Face / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009059:Mouth Diseases / Q000453:epidemiology / Q000209:etiology* / Q000473:pathology*; D010510:Periodontal Diseases / Q000453:epidemiology / Q000209:etiology / Q000473:pathology; D010513:Periodontal Ligament / Q000473:pathology; D012595:Scleroderma, Systemic / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D014987:Xerostomia / Q000453:epidemiology / Q000209:etiology / Q000473:pathology; D055815:Young Adult
keywords: Face; Oral; Oral medicine; Scleroderma; Sclérodermie; Sclérose systémique; Systemic sclerosis; Visage