A Case of Repeated Injuries Leading to a Diagnosis of Classical Ehlers-Danlos Syndrome

Abstract

Ehlers-Danlos syndrome is an umbrella term that includes a heterogeneous group of inherited connective tissue disorders. Classical Ehlers-Danlos syndrome is characterized by skin fragility, easy bruising, skin hyperextensibility, and joint hypermobility. Patients with this condition often develop wound dehiscence; however, limited information is available on wound care in these patients. This report describes the case of a 12-month-old boy who sustained a deep laceration on his right knee when he fell out of bed. Noting that the dermis was thinner and more fragile than normal, we performed suturing carefully with tension in two layers and waited for longer sutures. The wound took 4 weeks to heal but did so without unsightly scarring. However, the patient continued to present with recurrent injuries. Therefore, we performed a detailed examination, including molecular testing, and finally diagnosed the patient with classical Ehlers-Danlos syndrome. We identified several specific considerations that are important for wound management in classical Ehlers-Danlos syndrome.

Clinical photographs showing the laceration to the right knee.

(a) Photograph taken on the day of injury showing a 6-cm laceration with exposed subcutaneous tissue. (b) Photograph taken on day 8 after suturing. (c) Photograph taken on day 15 after removal of all sutures showing poor adhesion at some of the wound edges. (d) Photograph taken 2 months after suturing showing that the wound has healed without scarring.