Reduced strength of skin in osteogenesis imperfecta

The biochemical properties and ratio collagen type I/type III of skin biopsies from nine patients with osteogenesis imperfecta and nine age- and sex-matched controls were studied. Four of six patients with osteogenesis imperfecta Sillence type I had pronounced reductions in skin tensile strength, decreased ratios of collagen type I/type III, primarily accomplished by reduced amounts of collagen type I, moderate or no disability. The three patients with osteogenesis imperfecta Sillence type III had severe skeletal deformities, but normal skin tensile strength, and ratios of collagen type I/type III within the normal range. These observations may be explained as resulting from various structural defects in the type I collagen of patients with osteogenesis imperfecta.