Epidermolysis bullosa is a group of inherited skin fragility disorders with blister formation in the basement membrane zone. Chronic scarring after repeated blistering of the hands causes narrowing of the first web, flexion contractures of the digits, and pseudosyndactyly. Junctional epidermolysis bullosa is a type of epidermolysis bullosa that is characterized by blister formation in the lamina lucida. This condition is associated with survival into adulthood. In adult survivors, hand function might be required in order to participate in normal social activities. However, there is a lack of literature on the management of hand surgery for adult patients with junctional epidermolysis bullosa. We herein describe an adult patient with junctional epidermolysis bullosa who had pseudosyndactyly and flexion contractures in both hands. Five surgeries were performed. Contractures were released and reconstructed using split- or fullthickness skin grafts. Delayed wound healing was always observed due to epidermal necrosis in the graft. After epithelialization, a satisfactory functional outcome was obtained.
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Thomas Jefferson Univ, Sidney Kimmel Med Coll, Dept Dermatol & Cutaneous Biol, 833 Chestnut St,Suite 740, Philadelphia, PA 19107 USAThomas Jefferson Univ, Sidney Kimmel Med Coll, Dept Dermatol & Cutaneous Biol, 833 Chestnut St,Suite 740, Philadelphia, PA 19107 USA
Trufant, Joshua W.
Marzolf, Sean
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Med Univ S Carolina, Charleston, SC 29425 USAThomas Jefferson Univ, Sidney Kimmel Med Coll, Dept Dermatol & Cutaneous Biol, 833 Chestnut St,Suite 740, Philadelphia, PA 19107 USA
Marzolf, Sean
Leach, Brian C.
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Med Univ S Carolina, Charleston, SC 29425 USAThomas Jefferson Univ, Sidney Kimmel Med Coll, Dept Dermatol & Cutaneous Biol, 833 Chestnut St,Suite 740, Philadelphia, PA 19107 USA
Leach, Brian C.
Cook, Joel
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Med Univ S Carolina, Charleston, SC 29425 USAThomas Jefferson Univ, Sidney Kimmel Med Coll, Dept Dermatol & Cutaneous Biol, 833 Chestnut St,Suite 740, Philadelphia, PA 19107 USA
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Chaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, Israel
Chaim Sheba Med Ctr, Mohs Microg Surg Unit, IL-52621 Tel Hashomer, IsraelChaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, Israel
Zilinsky, Isaac
Farber, Nimrod
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Chaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, IsraelChaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, Israel
Farber, Nimrod
Weissman, Oren
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Chaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, IsraelChaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, Israel
Weissman, Oren
Israeli, Hadar
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Chaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, IsraelChaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, Israel
Israeli, Hadar
Haik, Josef
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Chaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, IsraelChaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, Israel
Haik, Josef
Domniz, Noam
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Chaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, IsraelChaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, Israel
Domniz, Noam
Winkler, Eyal
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Chaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, IsraelChaim Sheba Med Ctr, Dept Plast & Reconstruct Surg, IL-52621 Tel Hashomer, Israel