Autologous Transplantation of Cultured Fibroblast on Amniotic Membrane in Patients With Epidermolysis Bullosa

Epidermolysis Bullosa With Mitten Hands

Epidermolysis Bullosa mitten hand deformity autologous cultured fibroblast, amniotic membrane

Epidermolysis Bullosa (EB) is a blistering disease that is caused by defective anchoring fibrils and hemidesmosome in basement membrane of the skin layer. EB is inherited either autosomal or recessive and has 3 types. Recessive Dystrophic Epidermolysis Bullosa (RDEB) is severe with some morbidity such as mitten hand deformity. The management of these patients is very difficult because no effective treatment has been known yet. The EB patients with mitten hand deformity need surgery to have a biologic dressing for areas of hand which is without the skin. In this study the investigators assess the safety of autologous transplantation of cultured fibroblast on amniotic membrane (AM,as coverage) for them.

Epidermolysis Bullosa patients with mitten hand deformity were included. (based on inclusion & exclusion criteria) These patients should be offered for surgery to release the adhesions between fingers and MCP. When the consent form was accepted and the lab test was confirmed, a small skin biopsy was removed from retro auricle and fibroblast cells were separated and cultured. These cells were seeded on AM and the microbial test was done as evidence to prove the safety. Under general anesthesia, when the hands adhesion was removed, the autologous fibroblast cultured on AM was transplanted to the both hands (specially the area without skin coverage) and the hands were dressed afterward. The dressing was changed and the wound was checked regularly to detect any complications.

Iran