Wound Healing and Skin Grafting in Ehlers-Danlos Syndrome

Sir: The purpose of this article is to share our interesting and new experience in wound healing and skin grafting in patients with Ehlers-Danlos syndrome. A 22-year-old man, a known case of Ehlers-Danlos syndrome type IV, presented with ulceration over the right lower limb following trauma to the leg caused by a piece of wood 3 months previously. The wound was increasing progressively in size and was débrided at a local hospital before the patient was referred to us for further management. History of illness revealed closed reduction for congenital dislocation of the hip at 1 year of age, umbilical hernia that disappeared on conservative treatment, and rectal prolapse treated with sclerotherapy. He also had congenital bilateral microphthalmia with ptosis and ectopia lentis for which he was operated on at 18 years of age. He had a traumatic ulcer of the right leg 3 years previously, for which split-thickness skin grafting was performed. The grafted area healed in 20 days, whereas the thigh donor site took almost 2 months to heal. Local examination revealed a 15 × 10-cm ulcer on the anterior aspect of the middle third of the right leg, with sloughing and purulent discharge (Fig. 1). The wound bed was prepared in 3 weeks with limited access dressing,1 a combination of intermittent negative-pressure dressing, and moist wound healing. The wound was covered with a split-thickness skin graft from the left thigh, after which limited access dressing was reapplied. The total grafted area (measured by the graph paper method) was 472 cm2 and the total donor area was 440 cm2.Fig. 1.: Leg wound covered under limited access dressing.In the present case, limited access dressing was performed over both the donor area and the recipient area. Recipient area limited access dressing was removed after 10 days (99 percent take), and donor area limited access dressing was removed after 14 days (complete healing of the donor area). The exact prevalence of Ehlers-Danlos syndrome is unknown but is estimated to range from one in 10,000 persons to one in 25,000 persons, with Ehlers-Danlos syndrome type IV representing approximately 5 to 10 percent of all cases.2 Classic Ehlers-Danlos syndrome type IV is a heritable disorder characterized by joint hypermobility, skin hyperextensibility, abnormal wound healing, tissue fragility, and abnormal scar formation and wound healing.3,4 The majority of affected individuals have alterations in type V collagen genes, COL5A1 or COL5A2.3 In the present case, graft harvest with a Humby knife was difficult because of laxity of the skin, and the donor area at the beginning became deep at the usual setting for medium thickness grafts (Fig. 2). Normally, because of the primary contraction, a harvested graft shrinks by 9 percent as compared with the donor area produced.5 Contrary to this, the area of the intermediate thickness graft was 7 percent more than that of the skin graft donor area in our case. Although this patient had a history of delayed wound healing with conventional closed dressing on a previous occasion, his present wounds healed without any delay with intermittent negative-pressure dressing (limited access dressing).Fig. 2.: Deep portion of the donor area immediately after the start of harvest of the split skin graft. This problem was attributed to very lax skin.Pramod Kumar, M.S., M.Ch. Nitin Sethi, M.S., M.Ch. M. T. Friji, M.S., M.Ch. S. Poornima, M.B.B.S.