Surgical Management of Hand Contractures and Pseudosyndactyly in Dystrophic Epidermolysis Bullosa

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

“…Initially presenting as partial proximal web formation within the interdigital spaces of the hands and feet, progression may eventually lead to total encasement of the digits within a cocoon (''mitten'')-like keratinized sac, resulting in marked impairment of hand function and difficulties in weight bearing, standing, and walking. Earliest reports were based on case reports, 1,2 small case series, [3][4][5] or limited cohorts [6][7][8][9][10] of EB patients selected for and referred to specialized plastic and orthopedic surgical centers for reconstruction. In one of the more recently published series, involving 19 children between 2 and 14 years of age who were referred for hand surgery, 9 all had some degree of pseudosyndactyly and contracture; 12 of 19 had advanced cocoon formation.…”

“…Most of the earlier studies described degloving of the hands and immobilization of the digits, followed by grafting with either split-thickness grafts 4,7,13,14 or full-thickness skin. 6 Other variations on this technique have been subsequently described, to include the use of artificially prepared autologous skin graft sheets 5 or no grafts.…”

Inherited epidermolysis bullosa: Case report of finger localization

“…For many authors[710–12] skin grafts are useless in these patients even in severe cases because of the poor quality of the skin. They recommend “directed healing” after immobilization by Kirschner pins.…”

Novel and emerging therapies in the treatment of recessive dystrophic epidermolysis bullosa

“…This scarring leads to contractures and is most notable on the hands and feet (25). The changes affecting the hands include flexion contractures of the interphalyngeal joints, metacarpophalyngeal, and wrist joints.…”