Literature DB >> 16843123

Ehlers-Danlos syndrome type VIII: periodontitis, easy bruising, marfanoid habitus, and distinctive facies.

Megan M Moore1, Jennie M Votava, Seth J Orlow, Julie V Schaffer.   

Abstract

An 11-year-old boy had a history of easy bruising and poorly healing wounds since infancy and severe, early-onset periodontitis. He also exhibited mild hypermobility of the small joints of the hands, long limbs with striking arachnodactyly, and a triangular face with delicate features. Analysis of type I and type III collagens revealed no abnormalities. These findings were consistent with a diagnosis of Ehlers-Danlos syndrome type VIII (EDS-VIII), an autosomal dominant connective tissue disorder that was recently mapped to chromosome 12q13. We draw attention to the clinical features that typify EDS-VIII, including extensive pretibial bruising, a marfanoid body habitus, and characteristic facies, as well as childhood onset of progressive periodontal disease.

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Year:  2006        PMID: 16843123     DOI: 10.1016/j.jaad.2006.02.024

Source DB:  PubMed          Journal:  J Am Acad Dermatol        ISSN: 0190-9622            Impact factor:   11.527


  5 in total

1.  Ehlers-Danlos syndrome type VIII is clinically heterogeneous disorder associated primarily with periodontal disease, and variable connective tissue features.

Authors:  Eyal Reinstein; Celia Dawn DeLozier; Ziv Simon; Serguei Bannykh; David L Rimoin; Cynthia J Curry
Journal:  Eur J Hum Genet       Date:  2012-06-27       Impact factor: 4.246

2.  Early-onset osteoarthritis in Ehlers-Danlos syndrome type VIII.

Authors:  Eyal Reinstein; Mitchel Pariani; Ralph S Lachman; Stephen Nemec; David L Rimoin
Journal:  Am J Med Genet A       Date:  2012-03-14       Impact factor: 2.802

3.  Multiple odontogenic keratocysts in Ehlers-Danlos syndrome: a rare case report.

Authors:  Anna Starzyńska; Paulina Adamska; Łukasz Adamski; Aleksandra Sejda; Piotr Wychowański; Michał Studniarek; Barbara Alicja Jereczek-Fossa
Journal:  BMC Oral Health       Date:  2021-03-09       Impact factor: 2.757

4.  Ehlers-Danlos Syndrome Type VIII: A Rare Cause of Leg Ulcers in Young Patients.

Authors:  Sophie Ronceray; Juliette Miquel; Antoine Lucas; Gérald E Piérard; Trinh Hermanns-Lê; Anne De Paepe; Alain Dupuy
Journal:  Case Rep Dermatol Med       Date:  2013-10-03

5.  Fatal Ruptured Blood Blister-like Aneurysm of Middle Cerebral Artery Associated with Ehlers-Danlos Syndrome Type VIII (Periodontitis Type).

Authors:  Ulaş Cıkla; Alireza Sadighi; Andrew Bauer; Mustafa K Başkaya
Journal:  J Neurol Surg Rep       Date:  2014-08-11
  5 in total

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