Treatment of Severe Hand Deformities Caused by Epidermolysis Bullosa

Abstract

Little research has been done regarding the treatment of severe hand deformities caused by epidermolysis bullosa. A 14-year-old boy was diagnosed with congenital epidermolysis bullosa. He was treated in our hospital several times, but the pathogenetic condition worsened. On examination, both hands were clenched fists and had scar formation. Skin fusion was observed between the 5 fingers. Nails were absent and the thumb was in the fist. His fingers were short, and active and passive flexion and extension could not be performed. The right hand was treated first. After the adhesions were separated, we found that the 5 fingers were connected by dermis. After the dermis was separated and the hand was fixed in the extension position, there were small cutaneous deficiencies. The fingers were fixed in the functional position with Kirschner wires. The wound surface was covered with self-made aureomycin ointment gauze. After regular dressing changes for 6 weeks, the wound surface was completely healed. After 3 months of rehabilitation training, most hand function was recovered.Based on our findings in this case, when treating patients with epidermolysis bullosa, physicians must carefully observe whether enough dermis exists to avoid an unnecessary skin graft. We also found that the quality of skin used in skingrafting is questionable due to pathological changes in the skin. If there is enough dermis and the cutaneous deficiency is smaller after contracture release, the covering of drug dressings on the cutaneous deficiency is more conducive to the recovery of limb function and the reduction of damage to the donor sites.