Surgical management of hand deformity in epidermolysis bullosa: Initial experience and technique

Abstract

Epidermolysis bullosa describes a rare group of genetic mucocutaneous disorders characterized by excessive epithelial fragility resulting in mechanically induced blistering and abnormal wound healing.1,2 Its prevalence and incidence are 8.2 and 19.6 per 1,000,000 live births, respectively.2 Gene therapy, protein replacement, and cell therapy strategies have been investigated, but there is currently no cure.2 We describe a surgical technique to address the metacarpophalangeal joint flexion contracture and mitten deformity associated with EB.