Principles of Wound Care in Patients with Epidermolysis Bullosa

Lara‐Corrales, Irene; Arbuckle, Alan; Zarinehbaf, Sanaz; Pope, Elena

doi:10.1111/j.1525-1470.2010.01086.x

Cited by 21 publications

(18 citation statements)

References 45 publications

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“…It is relevant that extrinsic factors related to the HCT process itself could affect the skin healing. These include thorough wound care, immediate treatment of local and systemic infections (similar to the care people with RDEB receive at major EB centers [3,33]), protection from trauma due to relatively limited movement in the hospital room, and use of immune suppressive drugs to prevent graft-versus-host disease. Even though full immune reconstitution is only achieved many months after HCT, the administration of chemotherapy and immunosuppressive medications is limited to a week before and few months post-transplant, respectively.…”

Section: From Mouse To Manmentioning

confidence: 99%

“…Wound healing is a highly organized process that integrates the skin cells, skin extracellular matrix, and systemic factors-mainly blood cells and cytokines-into dynamic tissue healing [1][2][3][4]. Malfunction of the local arm (e.g., congenital deficiency of a structural skin protein in genodermatoses) or overwhelming of normal skin repair mechanisms (e.g., burn injury) or the systemic arm (e.g., breakdown of regulatory mechanisms that protect the skin from immune-mediated injury) can result in the loss of skin integrity and increased susceptibility to infections.…”

Section: Introductionmentioning

confidence: 99%

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Concise Review: Transplantation of Human Hematopoietic Cells for Extracellular Matrix Protein Deficiency in Epidermolysis Bullosa

2011

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The skin is constantly exposed to environmental insults and requires effective repair processes to maintain its protective function. Wound healing is severely compromised in people with congenital absence of structural proteins of the skin, such as in dystrophic epidermolysis bullosa, a severe congenital mechanobullous disorder caused by mutations in collagen type VII. Remarkably, stem cell transplantation can ameliorate deficiency of this skin-specific structural protein in both animal models and in children with the disorder. Healthy donor cells from the hematopoietic graft migrate to the injured skin; simultaneously, there is an increase in the production of collagen type VII, increased skin integrity, and reduced tendency to blister formation. How hematogenous stem cells from bone marrow and cord blood can alter skin architecture and wound healing in a robust, clinically meaningful way is unclear. We review the data and the resulting hypotheses that have a potential to illuminate the mechanisms for these effects. Further modifications in the use of stem cell transplantation as a durable source of extracellular matrix proteins may make this regenerative medicine approach effective in other cutaneous and extracutaneous conditions. STEM CELLS 2011;29:900-906Disclosure of potential conflicts of interest is found at the end of this article.

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Section: From Mouse To Manmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Concise Review: Transplantation of Human Hematopoietic Cells for Extracellular Matrix Protein Deficiency in Epidermolysis Bullosa

2011

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“…The blister roof should be left in place to facilitate re-epithelialization, to reduce infection risk and pain [10,31,32,42]. …”

Section: Care Of the Eb Newborn And Infantmentioning

confidence: 99%

Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa

Hachem

Zambruno

Bourdon-Lanoy

et al. 2014

Orphanet J Rare Dis

143

137

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BackgroundInherited epidermolysis bullosa (EB) comprises a highly heterogeneous group of rare diseases characterized by fragility and blistering of skin and mucous membranes. Clinical features combined with immunofluorescence antigen mapping and/or electron microscopy examination of a skin biopsy allow to define the EB type and subtype. Molecular diagnosis is nowadays feasible in all EB subtypes and required for prenatal diagnosis. The extent of skin and mucosal lesions varies greatly depending on EB subtype and patient age. In the more severe EB subtypes lifelong generalized blistering, chronic ulcerations and scarring sequelae lead to multiorgan involvement, major morbidity and life-threatening complications. In the absence of a cure, patient management remains based on preventive measures, together with symptomatic treatment of cutaneous and extracutaneous manifestations and complications. The rarity and complexity of EB challenge its appropriate care. Thus, the aim of the present study has been to generate multicentre, multidisciplinary recommendations on global skin care addressed to physicians, nurses and other health professionals dealing with EB, both in centres of expertise and primary care setting.MethodsAlmost no controlled trials for EB treatment have been performed to date. For this reason, recommendations were prepared by a multidisciplinary team of experts from different European EB centres based on available literature and expert opinion. They have been subsequently revised by a panel of external experts, using an online-modified Delphi method to generate consensus.ResultsRecommendations are reported according to the age of the patients. The major topics treated comprise the multidisciplinary approach to EB patients, global skin care including wound care, management of itching and pain, and early diagnosis of squamous cell carcinoma. Aspects of therapeutic patient education, care of disease burden and continuity of care are also developed.ConclusionThe recommendations are expected to be useful for daily global care of EB patients, in particular in the community setting. An optimal management of patients is also a prerequisite to allow them to benefit from the specific molecular and cell-based treatments currently under development.

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“…In this case, it might be that the dressing itself is not properly attached to the skin and is rubbing at the adhesive interface when the patient mobilises. This is especially true in patients with epidermolysis bullosa, who have extremely fragile skin (Lara-Corrales et al, 2010).…”

Section: Can Wound Dressings Cause Blisters and If So How?mentioning

confidence: 99%

A review of blisters caused by wound dressing components: Can they impede post-operative rehabilitation and discharge?

Eastburn

Ousey

Rippon

2016

International Journal of Orthopaedic and Trauma Nursing

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This review highlights that some wound dressings can be the cause of blistering. It also presents the mechanisms by which blisters may be caused by poor choice of dressings. The subsequent impact of the blisters on preventing patient mobility -and hence rehabilitation in terms of physiotherapy -are also identified. The possibility that the clinical sequelae (e.g. delayed wound healing, restricted joint range of motion (ROM), muscle atrophy and increased risk of deep vein thrombosis (DVT)) resulting from this might have a significant and deleterious impact upon patient-related outcomes is discussed. Strategies for the treatment and prevention of blisters are proposed, based upon current knowledge and expertise. The criticality of the wound care specialist and the physiotherapist working together to overcome these challenges and enhance patient care, are underlined. This article is a review of the relevant literature combined with opinions based upon experience and knowledge of the authors.

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Principles of Wound Care in Patients with Epidermolysis Bullosa

Cited by 21 publications

References 45 publications

Concise Review: Transplantation of Human Hematopoietic Cells for Extracellular Matrix Protein Deficiency in Epidermolysis Bullosa

Concise Review: Transplantation of Human Hematopoietic Cells for Extracellular Matrix Protein Deficiency in Epidermolysis Bullosa

Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa

A review of blisters caused by wound dressing components: Can they impede post-operative rehabilitation and discharge?

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