Ehlers-Danlos syndrome: classifications, oral manifestations, and dental considerations.
Ehlers-Danlos syndrome (EDS) is the name given for 6 types of connective tissue disorders. While the prevalence of this disease is small, it is seen on every continent and affects both sexes and all races. The various types of EDS are reviewed with reference given to both the older Berlin nosology and the newer Villefranche nosology. Phenotypes of EDS vary depending upon which type of collagen is altered, leading the practitioner to the diagnosis before biochemical confirmation is obtained. In this regard, because collagen is present throughout the head and neck, oral and maxillofacial manifestations of the disease are discussed and are readily noticeable to the astute dentist. Specialists in several fields of dentistry are made aware of the complications EDS can pose on treatment, healing, and follow-up care.
Abel
Mark D
MD
Oral & Maxillofacial Surgery, Hospital of the University of Pennsylvania, 5 White Building, 3400 Spruce St., Philadelphia, PA 19104, USA. mark.abel@uphs.upenn.edu
Carrasco
Lee R
LR
D004535:Ehlers-Danlos Syndrome / Q000145:classification* / Q000150:complications* / Q000235:genetics; D024022:Fibrillar Collagens / Q000235:genetics; D006801:Humans; D009056:Mouth Abnormalities / Q000209:etiology; D009154:Mutation; D006472:Oral Hemorrhage / Q000209:etiology; D019647:Oral Surgical Procedures / Q000009:adverse effects; D014071:Tooth Abnormalities / Q000209:etiology
D024022:Fibrillar Collagens