Ehlers-Danlos syndrome identified from periodontal findings: case report

Resident: Adam J. Bottrill
Date: 11MAR10
Region: Providence
Article title: Ehlers-Danlos syndrome identified from periodontal findings: case report
Author(s): Bond, P et al.
Journal: Pediatric Dentostry
Page #s: pp. 212-213
Year: 1993, May/June
Major topic: Ehlers-Danlos
Minor topic(s): Periodontal disease
Type of Article: Case Report
Main Purpose: Case report on type VIII Ehlers-Danlos patient with periodontal findings.
Overview of method of research: Case research

Key points in the article discussion:

I. Ehlers-Danlos: connective tissue disorder characterized by fragile skin, skin hyperextensibility, joint hypermobility. Type VIII was 1st described in 1972.
A. Autosomal dominant, post-pubertal periodontal disease, pretibial scarring,
B. NO joint hypermobility or skin hyperextensibility
C. Typically significant amount of overlap between type VIII and type IV (which can be fatal)

II. Case of HW
A. 7 yo white female with NSMH, NSDH, no FH.
B. Presents with parents “concerned about the appearance of her teeth.
C. Severe generalized gingivitis, recession and exposed root surfaces.
D. Radiograph revealed 4-5mm bony pockets.
E. Heavy calculus and premature exfoliating teeth.
F. Systemic disease suspected. (prolonged bleeding time, peritibial bruising)
G. Referal was made: EDS type VIII was Dx.

III. Discussion
A. Rare condition
B. DDX: histiocytosis X, cyclic neutropenia, Papillon-Lefevre, hypophosphatasia, leukemia, vit D resistant rickets, leukocyte disorders, acrodynia …. AND … EDS type VIII.
C. Because of possibility of EDS type IV and it’s potentially fatal prognosis, must include the EDS’s in DDX.

Assessment of article: Simple. Short. To the point… Semper Vigilans!!!