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.
De Felice
Claudio
C
Hospital-based Neonatologist and Clinical Researcher, Neonatal Intensive Care Unit, Azienda Ospedaliera Universitaria Senese, Siena, Italy.
Bianciardi
Giorgio
G
Dileo
Luigi
L
Latini
Giuseppe
G
Parrini
Stefano
S
D000293:Adolescent; D000328:Adult; D016022:Case-Control Studies; D004535:Ehlers-Danlos Syndrome / Q000473:pathology*; D005109:Extracellular Matrix; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009061:Mouth Mucosa / Q000002:abnormalities / Q000098:blood supply*; D023861:Photography, Dental