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, atrophic scars, and articular hypermobility. These features may have less important clinical presentation in the intermediate forms of type II EDS. The authors studied the ultrastructural and quantitative aspects of elastic and collagen 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 atrophic scars, articular hypermobility of the hands, and cutaneous and vascular 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 ultrastructural abnormalities were seen in diameter and curvature, such as torsion, collagen flower-like aspect and discrete mass enlargement by histophotometry.