Discussion
Fibro-osseous lesions begin with a central focus (nidus) of mineralization, mainly at the apical part of the teeth [11]. Here, initially replacing the spongy bone with fibrous tissue starts, followed by increasing mineralization [12]. CB-CT osteodensitometry allows the categorization of these changes and supports clinical monitoring [13, 14]. To find the best categorization and to separate these findings with different bone densities, clinicopathological findings from hyperparathyroidism-jaw tumor syndrome (HPT-JT), gnathodiaphysial dysplasia (GDD), and familial gigantiform cementoma (FGC) can be considered [15-20].
HPT-JT a genetic disorder associated with multiple parathyroid adenomas, renal tumors, and multiple ossifying fibromas. Blood investigation did not reveal changes in parathormone, nor did any co-morbidity show evidence [15]. GDD and FGC show phenotypical similarities, but patients with GDD often suffer from instability of long bones and bone fractures. Since there was no evidence of injuries in the very sporty women (BMI 21), this diagnosis was excluded [21]. Like HPT-JT and GDD also FGC is a systematic genetic disorder. At present, the biological mother, the deceased father’s sister, and a cousin on the father’s side have had a genetic panel examination. However, these relatives showed no evidence of pEDS or FGC. Some authors suggest that familial florid cemento-osseous dysplasia (COD) mimics FGC or represents different spectrums of the same clinicopathological process [11, 12]. Therefore, we excluded FGC by detecting Anoctamin 5 negative [7]. Further research and genetic studies are needed to improve the understanding and assess if conditions are indeed related.
So far, few authors report periapical manifestations in periodontal Ehlers-Danlos-Syndromes, as recently reviewed by Seebacher-Kapferer et al. (2020) [1]. Pulp calcification and pulp stones in classical Ehlers-Danlos-Syndrome was first described by Selliseth (1965) [22], confirmed by others [23, 24, 25]; We did not find atypical pulp-calcification in the remaining 24 teeth as described by Ferré et al. (2012) [26] or Majorana & Facchetti, [27] but drumstick-formation, of the molars, similar to the presenting case of a 41-year older woman described by Seebacher-Kapferer et al. (2020) [1]. The authors, as mentioned above explain, that calcification of the pulp is a common finding in cEDS, reported in 15/17 individuals, caused mainly by COL5A1 or COL5A2 and that irritations, decay, or chronic inflammations may induce pulp calcification. In the present case, the initial lesion appears to arise from the periapical vascular bundle. From which the nidus-like periapical mineralization derives as a secondary reaction. The pEDS usually leads to premature loss of teeth. The present case may show tooth-preservation and dentomaxillary development as a consequence of intensive dental hygiene. The biopsies revealed histological vascular proliferates in addition to bone sclerosis and fibrosis and confirmed the CB-CT-O findings.
It remains unclear whether the extent of periapical inflammation in the sense of microangiopathic processes can be understood. The authors assume that vascular changes could be a trigger for periapical cement dysplasia. The same consideration of a microangiopathic genesis would also make sense concerning the deterioration of vision, with unfortunately not detectable or objectify by ophthalmological investigation [28-31].