Adam Lang

Oral Systemic Link

Ehlers-Danlos Syndrome and Periodontitis Causality
A multitude of systemic conditions foster the etiology and progression of
periodontal disease, such as diabetes, cancer treatments, and viral infections.
There exists a multitude of inherited genetic disorders which also contribute to the
etiology and progression of periodontal disease through decreased modification of
humoral/cell mediated immunity. Maxillofacial anomalies, and mental handicap, in
relation to genetic disorders, provide limitations to a patients home care regimen.
Ehlers-Danlos syndrome envelopes unique modification of the host response, and
maxillofacial tissue structure integrity and their interplay to the progression of
periodontal disease.
Ehlers-Danlos syndrome is a group of clinical and genetic manifestations that
is characterized by connective tissue disorders. These disorders, were the
structural framework of the intraoral tissues, (extracellular matrixes) if deleteriously
affected by mutations in several types of collagen. Individual expression of this
heterogeneous X-linked disorder, contributes toward its level of penetrance, (or
clinical visibility of symptoms.) There exists no gender or racial/ethnic predilection,
and prevalence of EDS including its subtypes are 1:5000. (Wakhloo, 2015.)
Since the syndromes discovery in the early 1900s by a European
dermatologist and his colleague Henry Alexander Danlos. In the 1930s, hallmark
clinical and phenotypical features of the condition were compiled and the condition
was then named Ehlers-Danlos syndrome. Later in the 1980s, in Germany 10
subtypes were classified. In 1997 experts met to arrange the classification of six
subtypes. EDS inheritance was then termed to result from a single gene, and its
penetrance in a particular individual is based on Mendelian inheritance theory;
which demonstrates how genetic traits are passed from parent to offspring through
autosomal X-linked dominant or recessive expression. (Wakhloo, 2015.)
Wakhloo states the most prevalent intra oral indication in those with EDS, is
mucosal fragility. This is due, in part to basement membrane structural alteration
through collagen types, particularly the lamina propria which supports the oral
epithelium. (2015) Brushing and mastication contribute to mucosal tearing.
Mucogingival junctions, in particular the labial mucosa, are attached more coronally,
giving the patient a short upper lip appearance.

Early onset generalized periodontitis remains the chief clinical finding with
EDS VIII, (this subtype has the most dominant transmission and highest
penetrance.) A multitude of odontogenic tissue malformations such as abnormal
pulp shape, dentin scalloping, and supernumerary teeth serve as primary
contributing factors to periodontal disease. The landscape of the oral cavity hinders
home care hygiene difficult. Temporomandibular joint malformation contributes to
occasional dislocation of the joint. The prevalence of temporomandibular joint
disorder in individuals with EDS not only contributes to discomfort to the joint itself,
treatment options to resolve these issues are costly and not readily accessible.
Factors of TMD and its relationship to periodontal disease progression is due to
parafunctional habits such as night time bruxing. An EDL patient with existing
periodontitis may suffer secondary trauma from occlusion: Damage to teeth with
increased pressure on the PDL, which Nield-Gehrig states, contributes to additional
alveolar bone and increased eventual tooth loss. (p. 134-5, 2011)
The largest challenge in periodontally treating those with EDS is to reestablish soft tissue attachment. Wakhloo states collage fibers are more soluble,
and defective interdigitation or interlocking of said fibers to the root surface are
faulty. New collagen processing is absent in those with EDS, (2015.) Immunoloic
deficiencies in those with EDS include lymphocytopenia, normal CD-4/CD-8 ratios,
with natural killer cell count reduction.
Ascorbic acid with a dose of 1-4 grams, (above 100mg per day standard) is
recommended for those with EDS to foster collagen integrity. Administration of
NSAIDS and muscle relaxants to control myofascial pain. In type IV EDS, (vascular),
Wakhloo reports a prophylactic use of beta blockers is under research to control
congenital cardiovascular defects. (2015.) The RDH must be aware of continue
systemic effects of beta blockers on the periodontium.
Maxillofacial anomalies, and mental handicap, in relation to genetic disorders,
provide limitations to a patients home care regimen. Ehlers-Danlos syndrome
envelopes unique modification of the host response, and maxillofacial tissue
structure integrity and their interplay to the progression of periodontal disease.
Patients with EDS evoke a full-spectrum approach in developing an effective
treatment plan: TMD, occlusal disharmony, secondary tooth trauma, home care
product selection and custom home-care education.
Wakhloo, T, & Kiran, A., (2015). Ehlers-Danlos syndrome. Journal of Dental
Research and

Review, 2:1. DOI: DOI: 10.4103/2348-2915.154652