Surgery, compression bandage aid RDEB woman’s deformed hand
Case report describes correction of finger adhesion, contractures
Surgical correction followed by continuous compressive bandaging effectively treated hand finger adhesion and contractures in a woman with recessive dystrophic epidermolysis bullosa (RDEB), according to a report.
RDEB patients commonly undergo hand surgeries to improve function, and may require orthoses post-surgery to preserve results. Patients usually need several procedures.
In this case, compressive bandaging prevented the need for specialized orthoses, and no reoperation was needed. “Continuous bandaging preserves range of motion and delays deformity recurrence, proving beneficial for RDEB patients,” the researchers wrote.
The case was described in a letter to the editor, “Postoperative continuous compression bandaging was a useful technique for improving pseudosyndactyly in recessive dystrophic epidermolysis bullosa patients,” published in The Journal of Dermatology.
Surgeries for deformed hand
DEB is caused by mutations in the COL7A1 gene, which encodes a portion of collagen VII, a protein that connects skin layers. In recessive DEB, patients must inherit two copies of disease-causing mutations to develop the disease.
When collagen VII is faulty or missing, skin connections are fragile, and even small movements may trigger the formation of skin blisters and subsequent scar tissue. When occurring in the hands and feet, it may lead to pseudosyndactyly (fusion) and contractures (when muscles, tendons, joints, or other tissues tighten or shorten, causing a deformity).
Researchers at the University of Miyazaki, Japan, described the case of a 36-year-old woman with recessive DEB referred for pseudosyndactyly of the left hand. She had undergone four surgeries in the previous three years.
The researchers decided to perform another surgery, which began by removing the affected epidermis, the skin’s outermost layer, releasing the contractures. After exposing the dermis, they used artificial skin grafts to cover the palm and fingers. They then applied gauze soaked in polymyxin B, an antibiotic, to prevent skin infection. This also helped maintain a wet-to-dry environment and non-adherent dressing. A boxing glove-like dressing was also used to help with wrist flexion and extend finger joints.
After five days, the silicon film from the artificial grafts was removed, and the hands were covered with a “non-adherent vaseline gauze” with an alginate-coated dressing placed over it and between the fingers. Alginate, when in contact with wound fluid, forms a firm, moist gel that is highly absorbent.
“Continued dressing changes maintained a good condition,” and the complete formation of a new epidermis layer took over nine weeks, the researchers wrote. After recovery, the woman was satisfied with the results and saw an improvement in hand use for daily activities. At a follow-up evaluation three years and eight months after the surgery, no adhesion of fingers was noted.
About the Author
