'downhill' extremely rapidly. The majority of patients do not survive beyond 30 years.
The progressive periodontal condition is very difficult to control and is often of
secondary importance to other life-threatening infections.
11.12.5 Ehlers-Danlos syndrome
The syndrome is an autosomal-dominant trait with nine variants that display defects in
the synthesis, secretion, or polymerization of collagen. The variants of the syndrome
exhibit extensive clinical heterogeneity and collectively represent the most common
of the heritable disorders of connective tissues. The clinical findings are principally
excessive joint mobility, skin hyperextensibility, and susceptibility to scarring and
bruising of the skin and oral mucous membranes (640HFig. 11.16). Defective type IV
collagen supporting the walls of small blood vessels predisposes to persistent
postextraction haemorrhage.
Gingival tissues are fragile and have a tendency to bleed on toothbrushing. The type
VIII syndrome is associated with advanced periodontal disease. Periodontitis has also
been linked with the type IV variant, although other variants do not appear to be
affected. Ultrastructural changes also occur in the teeth, with abnormalities of the
amelodentinal junction, vascular inclusions in dentine, fibrous degeneration of the
pulp, and disorganization of cementum.
Type VIII patients require a thorough preventive periodontal programme as root
debridement can cause extensive trauma to the fragile soft tissues. Periodontal surgery
should be avoided because of the risk of haemorrhage and the potential problems
encountered with suturing soft tissue flaps.
641H
Fig. 11.16 Cutaneous hyperextensibility
of the upper eyelids in a 9-year-old child
with Ehlers-Danlos syndrome.