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].