Ehlers-Danlos Syndrome and Skin Grafting

Ehlers-Danlos syndrome is a heritable connective tissue disorder with an estimated prevalence of one in 10,000 to 25,000 persons [1]. Using clinical phenotype, biochemical and molecular defects, as well as inheritance patterns, a total of 6 subtypes have been described [2]. Although its hallmark findings include severe joint laxity and hyperextensible skin, this condition is known to affect a variety of different organ systems, including the cardiovascular, gastrointestinal, and bronchopulmonary systems [3]. The underlying disorder is related to alterations of collagen type I, III, and V resulting in architectural changes and decreased cross-linking of collagen fibers [4]. This translates into decreased breaking strength of collagen with its clinical correlates being skin fragility, easy bruising, and susceptibility to minor trauma [3]. The concern for surgeons is primarily related to bleeding and poor wound healing, as patients with Ehlers-Danlos syndrome tend to develop wound dehiscence and delayed wound healing postoperatively [5,6].