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Review
. 2022 Nov 7;17(1):406.
doi: 10.1186/s13023-022-02282-0.

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

Rachel Box  1 , Catina Bernardis  2 , Alexander Pleshkov  3 , Nicky Jessop  4 , Catherine Miller  5 , Jennifer Skye  6 , Virginia O'Brien  6 , Matthew Veerkamp  7 , Anna Carolina Ferreira da Rocha  8 , Roger Cornwall  9
Affiliations
Review

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

Rachel Box et al. Orphanet J Rare Dis. .

Erratum in

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.

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Conflict of interest statement

One CPG panel member declared a conflict of interest with respect to this guideline; however, following their resignation, their involvement ceased after the searches and first panel meeting, therefore they have not influenced the recommendations or outcomes of this guideline. The remaining CPG panel members and reviewers declared no conflicts of interest with respect to the publication of this guideline.

Figures

Fig. 1
Fig. 1
Hand wraps dorsal view
Fig. 2
Fig. 2
Hand wraps volar view
Fig. 3
Fig. 3
Skinnies™ dressing glove volar view
Fig. 4
Fig. 4
Skinnies™ dressing glove dorsal view
Fig. 5
Fig. 5
Skinnies™ Reinforced web-spacer glove
Fig. 6
Fig. 6
Soft Putty Elastomer™ web space splint volar view
Fig. 7
Fig. 7
Bilateral Soft Putty Elastomer™ web space splints dorsal view
Fig. 8
Fig. 8
Thermoplastic splint for a child
Fig. 9
Fig. 9
Thermoplastic splint for an adult
See this image and copyright information in PMC

References

    1. UK D. National charity that supports individuals and families affected by epidermolysis bullosa. https://www.debra.org.uk (2021).
    1. Intong LRA, Murrell DF. Inherited epidermolysis bullosa: new diagnostic criteria and classification. Clin Dermatol. 2012;30:70–77. doi: 10.1016/j.clindermatol.2011.03.012. - DOI - PubMed
    1. Bernardis C, Box R. Surgery of the hand in recessive dystrophic epidermolysis bullosa. Dermatol Clin. 2010;28:335–341. doi: 10.1016/j.det.2010.01.013. - DOI - PubMed
    1. Vozdvizhensky SI, Albanova VI. Surgical treatment of contracture adn syndactyly of children with epidermolysis bullosa. Br J Plast Surg. 1993;46:314–316. doi: 10.1016/0007-1226(93)90010-9. - DOI - PubMed
    1. International D. Epidermolysis Bullosa (2019).

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