Rehabilitation in Recessive Dystrophic Epidermolysis Bullosa

Pajardi, G.; Pivato, G.; Rafanelli, G.

doi:10.1097/00130911-200109000-00009

Cited by 5 publications

(10 citation statements)

References 4 publications

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“…Articles made no specific comments about surgeon experience, this ranged from an expert reporting on 45 individuals (80 hands adults and children) operated on by a single surgeon, to a number of Case Reports or small series of 2–4 individuals [ 7 , 11 , 28 , 37 , 46 , 47 ]. Interestingly, results for ‘recurrence’ were best for the expert surgeon, with maintenance of thumb adduction for 2 years; improvement in flexion contractures and pseudosyndactlyly up to 5 years [ 7 ].…”

Section: Resultsmentioning

confidence: 99%

“…We recommend post-operative hand orthoses/splints are worn to maintain the surgical gains and help delay contracture recurrence, supported by most articles [3,11,18,19,24,27,29,38,45,47]. This should start as soon as individuals can tolerate the fabrication process, which may be while the hand is still fully dressed [19,45].…”

Section: General Principlesmentioning

confidence: 98%

“…The type of skin graft used is dependent on whether operating on children or adults, whole hand or only the first web space is released. Thumb contribution to hand function is well known and first webspace deemed important: several authors used FTSG here [42,47,49].…”

Section: Hand Surgerymentioning

confidence: 99%

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Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

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Bernardis

Pleshkov

et al. 2022

Orphanet J Rare Dis

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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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Section: Resultsmentioning

confidence: 99%

Section: General Principlesmentioning

confidence: 98%

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Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

Box

Bernardis

Pleshkov

et al. 2022

Orphanet J Rare Dis

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“…Composite cultured skin (CCS) has been used in the treatment for donor sites and mitten‐like deformities in EB, but clinical trials are still going on . Also amniotic membrane grafts, epidermal keratinocyte grafts and different splints have been used in the treatment for EB with hand deformities .…”

Section: Discussionmentioning

confidence: 99%

Suprathel^®‐assisted surgical treatment of the hand in a dystrophic epidermolysis bullosa patient

Sarı

Eryılmaz

Tetik

et al. 2014

International Wound Journal

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Epidermolysis bullosa (EB) is a progressive familial disorder composed of dermal mucosal blisters, flexion contractures and pseudosyndactylies. Flexion contractures and pseudosyndactyly can be treated with surgery but usually require skin grafting. Because of poor wound healing, skin graft harvesting is a challenge in these patients. In order to prevent donor-site morbidities due to skin graft harvesting some alloplastic materials were introduced. In this study, we focused on Suprathel(®) as a new allograft material for covering the skin defects of a patient with dystrophic EB.

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“…The most appropriate dressing type depends on an individual's EB subtype, the characteristics and location of the wound. [13][14][15][16][17] EB patients need dressings that are nonadherent, semi-permeable and absorbent. 15 Dressing use is also determined by patients' preferences and practitioner experience 15,24,25 however dressing changes are often painful and time consuming.…”

Section: Introductionmentioning

confidence: 99%

Iterative codesign and testing of a novel dressing glove for epidermolysis bullosa

et al. 2019

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Background: Recessive Dystrophic Epidermolysis Bullosa is a rare genetic skin disorder which requires intensive hand therapy to delay fusion of the digits. Existing dressings do not conform to the complex structure of the hand and are applied in patches held with additional bandages leading to an occlusive environment. Objectives: To co-design a dressing glove based on user experiences and needs. Methods: Qualitative interviews and focus groups with children and adults with Recessive Dystrophic Epidermolysis Bullosa and their carers were conducted. Iterative feedback of design cues, bench and surrogate testing of materials and prototype refinement were achieved through collaborative co-design with patients, carers, clinicians and manufacturers. Results: Thematic analysis generated eight user needs and corresponding design cues addressing issues of absorbency, adherence, comfort, adaptability, ease of application and removal, breathability, protection, and hand hygiene. One prototype was selected for proof of concept testing. Conclusion: This novel dressing glove design meets the patient's requirements of a dressing, which conforms to the structure of the hand and sits into the web spaces to keep fingers separated. Proof of concept testing has since been undertaken with patients to determine performance, value for money and whether further developments are required and will be published in a separate paper.

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Rehabilitation in Recessive Dystrophic Epidermolysis Bullosa

Cited by 5 publications

References 4 publications

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

Suprathel^®‐assisted surgical treatment of the hand in a dystrophic epidermolysis bullosa patient

Iterative codesign and testing of a novel dressing glove for epidermolysis bullosa

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Rehabilitation in Recessive Dystrophic Epidermolysis Bullosa

Cited by 5 publications

References 4 publications

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa

Suprathel®‐assisted surgical treatment of the hand in a dystrophic epidermolysis bullosa patient

Iterative codesign and testing of a novel dressing glove for epidermolysis bullosa

Contact Info

Product

Resources

About

Suprathel^®‐assisted surgical treatment of the hand in a dystrophic epidermolysis bullosa patient