Abnormal oral vascular network geometric complexity in Ehlers-Danlos syndrome

Ehlers-Danlos syndrome (EDS) leads to impaired extracellular matrix (ECM) structure. Since ECM plays a major role in blood vessel geometry, we tested the hypothesis of an abnormal geometrical complexity of the visible microvascular network in EDS. Twelve patients with EDS-II or EDS-III and a control population (n=12) were examined. The geometry characteristics for the networks on the lower vestibular oral mucosa were analyzed using high-resolution photographic images. Fractal dimension, D, at 2 scales (D 1-46 and D 1-15), blood vessel tortuosity (minimum-path fractal dimension , Dmin ), and relative Lempel-Ziv complexity (L-Z) values were determined. EDS vascular networks exhibited significantly higher D 1-46 (P <.00001) and D 1-15 (P <.00001), as well as L-Z complexity (P <.00001), together with lower Dmin values (P=.0001) than controls. These findings indicate the presence of a previously unrecognized microvascular network abnormality on the oral mucosa in EDS patients, and provide an additional phenotypical marker for the condition.