Ehlers-Danlos syndrome, type VIII presenting with periodontitis and prolonged bleeding time

Clin Dysmorphol. 1995 Apr;4(2):145-9.

Abstract

We report on a case of Ehlers-Danlos syndrome, probable type VIII (EDS VIII) in a 6-year-old girl with severe periodontitis, extensive bruising of the shins, abnormal bleeding time, and thin body habitus. The structure and biosynthesis of types I and III colagen were normal. Desmopressin (DDAVP) was found to correct the bleeding time, and the patient underwent an uneventful dental procedure after DDAVP therapy. The finding of childhood or juvenile periodontitis should prompt consideration of a diagnosis of EDS, particularly type VIII, and alert the clinician to the possibility of a treatable bleeding abnormality.

Publication types

  • Case Reports

MeSH terms

  • Blood Coagulation Disorders / drug therapy
  • Blood Coagulation Disorders / physiopathology*
  • Child
  • Deamino Arginine Vasopressin / therapeutic use
  • Ehlers-Danlos Syndrome / physiopathology*
  • Female
  • Humans
  • Periodontitis / physiopathology*
  • Time Factors

Substances

  • Deamino Arginine Vasopressin