Orphanet Journal of Rare Diseases (Nov 2022)

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

  • Rachel Box,
  • Catina Bernardis,
  • Alexander Pleshkov,
  • Nicky Jessop,
  • Catherine Miller,
  • Jennifer Skye,
  • Virginia O’Brien,
  • Matthew Veerkamp,
  • Anna Carolina Ferreira da Rocha,
  • Roger Cornwall

DOI
https://doi.org/10.1186/s13023-022-02282-0
Journal volume & issue
Vol. 17, no. 1
pp. 1 – 21

Abstract

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Abstract What is already known about this topic? Epidermolysis bullosa (EB) causes blistering and scarring of the hands resulting in contractures fused web spaces and altered function. Surgery is needed to release contractures and web spaces and hand therapy is essential to maintain results, approaches for both differ. What does this study add? These guidelines aim to provide information on the surgical and conservative therapeutic hand management of children and adults diagnosed with EB. They are based on available evidence and expert consensus to assist hand surgeons and therapists in decision making, planning and treatment. They highlight the importance of a holistic multidisciplinary team (MDT) approach, where patient priorities are paramount.