Case report: Multiple ICRR a Co-Incident with Progeria Syndrome

Objectives: Hutchinson Guilford progeria syndrome (HGPS) is a rare and fatal “premature aging” disease which caused by a single mutation and results in the production of a mutant protein "Progerin". Its dental manifestations include abnormal tooth formation, hypodontia, micrognathia and delayed eruption. We present a 18-year-old HGPS patient, with mixed dentition due to over-retained teeth and crowding. All primary teeth were extracted, maxillary and mandibular premolars were partially erupted and maxillary canines were impacted. Cone Beam Computerized Tomography (CBCT) obtained and 7 lesions of Invasive Cervical Root Resorption (ICRR) were observed. ICRR lesion is characterized by a resorptive process which leads to a destructive loss of tooth structure. This report aimed to understand the biological association between HGPS and ICRR.
Methods: 50 articles regarding Progeria and ICRR were reviewed in order to find the mechanism between these phenomena.
Results: ICRR etiology includes trauma, bleaching, surgery, inflammation and orthodontic treatment. However in this case of progeria the 7 ICRR lesions were diagnosed prior to any kind of dental treatment and were not a direct consequence of an early dental treatment. Progeria patients may become more vulnerable by exhibiting structural changes in cementum's inorganic material. Moreover, they exhibit immunological alterations such as an increased in β lymphocytes and NK-cell number which can lead to an immune response accelerated ICRR lesions.
Conclusions: HGPS patients tend to multiple ICRR lesions. The combination of a structural changes and a strong immune response may explain the association. Further investigation in this field is warranted.