[Absence of inferior labial or lingual frenula in Ehlers-Danlos syndrome: a new diagnostic criterion?].

BACKGROUND

Ehlers-Danlos syndrome comprises a rare and heterogeneous group of diseases affecting the skin, joints and vessels. Diagnosis is based on clinical score since molecular testing is not routinely available. High sensitivity and specificity concerning the absence of inferior labial and lingual frenula has been reported in Ehlers-Danlos syndrome, but the diagnostic value of this finding has been contested.

PATIENTS AND METHODS

We here report the cases of 2 female patients, one with a vascular form of Ehlers-Danlos syndrome complicated by spontaneous dissection of the carotid and vertebral arteries, and the other with classic type; in both women, inferior labial and lingual frenula were absent.

DISCUSSION

We discuss the discrepancies between the 3 published studies dealing with abnormalities of the inferior labial and lingual frenula in Ehlers-Danlos syndrome. We suggest routine oral examination in patients with suspected Ehlers-Danlos syndrome. The diagnostic value of absence of oral frenula in Ehlers-Danlos syndrome requires confirmation by a prospective study.