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

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.