Reduced Strength of Skin in Ehlers Danlos Syndrome, Type III

Abstract

The biochemical properties, ratio collagen type I/type III and the pattern of reducible collagen crosslinks were studied in skin biopsy samples from 10 patients with Ehlers Danlos syndrome type III (ED III) and 10 age-and sex-matched controls. The ED III patients had marked reductions in skin strength and stiffness (42% and 22%, respectively). The ultimate extensibility of the skin, however, was not different from that of the controls. Consequently, a certain load applied to the skin of these patients results in greater deformation than in that of the controls, due to reduced stiffness. The bursting limit is no different from that of the controls. Thus the skin in the ED III patients is not hyperextensible, it merely has reduced strength and stiffness. The reduction in strength could not be explained by differences in skin thickness or collagen content. Skin thickness, collagen content and ratio collagen type I/type HI were the same as in the controls, as also was the pattern of reducible collagen crosslinks. These alterations in the mechanical properties of the skin of patients with ED III may be attributable to the molecular structure of the mature collagen or the arrangement of the collagen fibres.

Key Words:

• collagen
• Ehlers Danlos syndrome type III
• connective tissue diseases
• hereditary disease