Clinical and functional outcomes of hand surgery for recessive dystrophic epidermolysis bullosa.

Abstract

Recessive dystrophic epidermolysis bullosa causes blistering and scarring of the hands, resulting in contractures fused web spaces and altered function. The aim of this study was to present the short- and mid-term clinical and functional results of a large case series of patients who underwent surgery for recessive dystrophic epidermolysis bullosa-related pseudosyndactyly. The study included 75 hands in 39 children (21 boys, 18 girls) with a mean age of 8 years (range, 4-15) and minimum follow-up of 1 year. Children were assessed with the Birmingham Epidermolysis Bullosa Severity Score Sheet and the ABILHAND-Kids questionnaire before operation and at final follow-up. There were varying degrees of recurrent scarring in all the hands by 24 months after operation. The ABILHAND-Kids questionnaire showed a significant improvement in ability to perform 17 of 21 tasks. In conclusion, despite recurrence that may develop in the short and mid term, surgical release can provide significant improvements in hand function.Level of Evidence: IV.