The ultrastructural and histophotometric study of elastic and collagen fibers in type II Ehlers-Danlos Syndrome and subclinical forms

Skin disorders in type II Ehlers-Danlos Syndrome (EDS) are characterized by signs of cutaneous hyperdistensibility, skin and vascular fragility, atrop hic scars, and articular hypermobility. These features may have less import ant clinical presentation in the intermediate forms of type II EDS. The aut hors studied the ultrastructural and quantitative aspects of elastic and co llagen fibers in the skin of individuals with subclinical signs of type II of Ehlers-Danlos Syndrome. A group of 27 individuals (Group I) with large a trophic scars, articular hypermobility of the hands, and cutaneous and vasc ular fragility were compared with 10 healthy individuals. The subjects from both groups were volunteers from Hospital das Clinicas da Universidade de Sao Paulo. The elastic fibers did not show alterations but collagen ultrast ructural abnormalities were seen in diameter and curvature, such as torsion , collagen flower-like aspect and discrete mass enlargement by histophotome try.