BMC Oral Health (Mar 2021)

Multiple odontogenic keratocysts in Ehlers–Danlos syndrome: a rare case report

  • Anna Starzyńska,
  • Paulina Adamska,
  • Łukasz Adamski,
  • Aleksandra Sejda,
  • Piotr Wychowański,
  • Michał Studniarek,
  • Barbara Alicja Jereczek-Fossa

DOI
https://doi.org/10.1186/s12903-021-01472-9
Journal volume & issue
Vol. 21, no. 1
pp. 1 – 7

Abstract

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Abstract Background An odontogenic keratocyst is a lesion characterized by aggressive and infiltrative growth. The lesion is characterized by the existence of satellite microcysts (microtumours) and frequent recurrence (up to 30%). Ehlers–Danlos syndrome is a condition in which collagen production or its post-translational modifications are affected. Defects in connective tissues cause symptoms, which range from mild joint hypermobility to life-threatening complications. Case presentation We present an extremely rare case of an 11-year old girl with Ehlers–Danlos syndrome and coexistence of multiple odontogenic keratocysts. Conclusions This case shows mainly atypical or rare association between multiple odontogenic keratocysts and Ehlers–Danlos syndrome.

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