Burn Injury and Wound Healing in X-Linked Ichthyosis

Abstract

X-linked ichthyosis is a skin condition of decreased keratin degradation and hyperkeratosis resulting from a deficiency of steroid sulfatase causing scaly skin. Burns in these patients may require skin grafting and harvesting from diseased donor sites. No descriptions of the outcomes of attempted grafting, donor site healing, and burn recovery in patients with X-linked ichthyosis exist. The authors describe split-thickness skin grafting in one patient with X-linked ichthyosis who sustained a burn with crush injury to his bilateral lower extremities. Although he developed cellulitis, there is no evidence that patients with ichthyosis have higher rates of infection. The patient exhibited rapid healing at postgrafting clinic visits with a much flatter texture than expected early after meshed skin grafting. This could be a benefit of the excess keratin state. Wound healing was not impaired by the ichthyosis. Concerns over skin harvest were alleviated by aggressive topical emollients, which did not negatively impact harvest of donor skin or primary burn site healing.