Ehlers-Danlos syndrome, type VIII presenting with periodontitis and prolonged bleeding time.
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.
Cunniff
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C
Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, USA.
Williamson-Kruse
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L
D001778:Blood Coagulation Disorders / Q000188:drug therapy / Q000503:physiopathology*; D002648:Child; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D004535:Ehlers-Danlos Syndrome / Q000503:physiopathology*; D005260:Female; D006801:Humans; D010518:Periodontitis / Q000503:physiopathology*; D013997:Time Factors
D003894:Deamino Arginine Vasopressin