Dental histology in familial expansile osteolysis

Abstract

The dental histopathology is described of patients suffering from Familial Expansile Osteolysis (FEO), a rare and unique autosomal dominant disease which has severe bony as well as dental consequences. The predominant dental features are resorption of the cervical and apical regions of the roots, premature depositions both of irregular secondary dentin, which causes narrowing of the pulp cavities, and of cellular cementum, which causes patchy narrowing of the periodontal ligament. The dental pulp shows age-like changes, including multiple pulp stones and the ligament vasculature appears to be abnormal. Evidence for involvement of enamel is equivocal. This combination of dental effects together with the bony lesions distinguishes the disease from idiopathic external resorption of either the single or multiple kind, despite similarities in the histopathology of the resorptive lesions; and from other non-hereditary and hereditary osteolytic diseases.

Benign Fibro-Osseous Lesions of the Craniofacial Complex A Review

“…Familial expansile osteolysis (FEO) was first described in a family from Northern Ireland and is inherited as an autosomal dominant trait [87][88][89][90][91]. The disease becomes manifest in the second decade and is characterized by osteoclastic resorption with cancellous bone expansion and elevation in serum alkaline phosphatase.…”

Progressive, Generalized, Apical Idiopathic Root Resorption and Hypercementosis

“…The possibility of heredity having a role in multiple root resorptions has been mentioned in the literature, [23][24][25] but in this case possible genetic factors could not be ascertained. The patient's father was examined radiologically and exhibited generalized chronic periodontitis with no signs of root resorptions.…”

“…In some cases, a familial pattern was noted. [23][24][25] Root resorption can also occur without any determined intrinsic or extrinsic cause, and in these situations, the term idiopathic is applied. Consequently, idiopathic root resorption remains an appropriate term to be used when the condition exists without a known etiology.…”

The role of systemic conditions and disorders in periodontal disease

“…Several other genetic diseases can lead to destruction of the periodontal tissues. These include familial expansile osteolysis (98) and familial hypophosphatemic vitamin D-resistant rickets. Familial vitamin Dresistant rickets differs from other forms of rickets in children and osteomalacia in adults, which are dietrelated and can be treated by supplying adequate calcium phosphate in the diet and also by administering vitamin D (106).…”