Epidermolysis Bullosa With Mitten Hands Clinical Trial
Official title:
Autologous Transplantation of Cultured Fibroblast on Amniotic Membrane for Mitten Hand Deformity in Patients With Epidermolysis Bullosa
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.
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Endpoint Classification: Safety Study, Intervention Model: Single Group Assignment, Masking: Open Label, Primary Purpose: Treatment