Surgical Correction of Syndactyl Deformities of Hand and Feet in Epidermolysis Bullosa-A Case Report

Abstract

Background: Epidermolysis bullosa (EB) is a rare disease that is usually inherited, begins early in life, and results in bullae and erosion at sites of trauma, usually on the hands (over joints), elbows, knees, and feet. Aim and Objectives: The primary goal of surgical treatment is to save life and improve the function of the involved extremities through surgical reconstruction of deformities and contractures. Materials and Methods: We report a case of a 2-month-old girl with eroded skin over both hands and feet since birth. The skin lesions progressed with numerous grouped bullae, and erosions on a noninflamed base on the palms and soles which resulted in syndactyl deformities and flexion contracture of hands and feet. Results: After serial surgical procedures including web release, contracture release, local flap transposition and full thickness skin graft, the patient regained useful hand function and an acceptable appearance of both feet. We report our experience of surgical reconstruction of hands and feet due to EB.