Advances in epidermolysis bullosa hand deformity management: a 20-year journey of techniques and outcomes
DOI:
https://doi.org/10.2340/jphs.v61.45064Keywords:
Epidermolysis, bullosa, blisters, pseudosyndactylyAbstract
Introduction: Epidermolysis bullosa (EB) is a rare disease involving the skin with the formation of synechiae and cicatricial retractions, especially on the hands.
The objective of this study is the evaluation of the surgical technique of debridement of the amorphous hand in patients suffering from EB.
Material and methods: All patients were treated according to the ethical principles of the Helsinki declaration. Data collection was performed in terms of patient’s hospitalization time, healing time (healing for more than 90% of the epidermis), possible recurrence and reoperation times, Visual Analogue Scale (VAS) for pain evaluation, and Disability of Arm-Shoulder-Hand (DASH) score in preoperative time and after 60 days from surgery. The clinical follow-up ranges from a minimum of 36 months up to 10 years.
Results: Complete reepithelialization of the hands (<90% of the bloody surface) occurred in 19.2 days, with a range of 14–23 days. In our study, the improvement in the DASH score and pain evaluation with VAS scale were statistically significant (P < 0.05), and the goal of this treatment was the functional recovery of the hands with resumption of daily autonomy.
Conclusion: The surgical technique shown is safe, simple, and repeatable with a rapid learning curve, and no complex instruments or large operating spaces are needed, which is why it should be easy to make it repeatable in other plastic or hand surgery centers. Our data regarding discomfort and the DASH scale give us encouraging premises to continue in this direction.
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Copyright (c) 2026 Francesco Ciancio, Antonio Cuzzocrea, Caterina Alberti, Dario Melita, Paolo Marchica, Rosario Ranno, Giuseppe A.G. Lombardo
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