Molecular genetic and clinical review of Ehlers–Danlos Type VIIA: implications for management by the plastic surgeon in a multidisciplinary setting

Summary A syndrome now known as Ehlers–Danlos, comprising laxity and fragility of the skin associated with hypermobility of the large joints, was published in 1892 by Tschernogobow. Ehlers–Danlos type VIIA is an extremely rare form of the syndrome. While the UK-based Ehlers–Danlos Support Group® recommends that the surgical management of patients with Ehlers–Danlos VIIA should be carried out in conjunction with a plastic surgeon, there is nothing in the plastic surgery literature regarding this syndrome. The management of patients suffering from Ehlers–Danlos VIIA is highly complex, as a result of the breadth of genetic and phenotypic presentations, and resulting complications. We present a review of the literature regarding this syndrome and, in particular, the surgical problems that may be encountered. A case report outlining our experience of successfully managing this condition is also presented.