scholarly journals Limited Hand Surgery in Epidermolysis Bullosa

10.5772/33631 ◽  
2012 ◽  
Author(s):  
Bartlomiej Noszczyk ◽  
Joanna Jutkiewicz-Sypniewsk
Keyword(s):  
Epidermolysis Bullosa ◽  
Hand Surgery

scholarly journals New protocol treatment of pseudo-syndactyly in the Epidermolysis Bullosa hand using the dermal regeneration template glove

2020 ◽  
Author(s):  
Fedele Lembo ◽  
Domenico Parisi ◽  
Liberato Roberto Cecchino ◽  
Francesco Ciancio ◽  
Alessandro Innocenti ◽  
...  
Keyword(s):  
Hospital Stay ◽  
Epidermolysis Bullosa ◽  
Hand Surgery ◽  
Complete Healing ◽  
Long Term Results ◽  
Minimal Trauma ◽  
Stay Time ◽  
Dermal Regeneration Template ◽  
Microsurgical Approach ◽  
Dermal Regeneration

Abstract BACKGROUNDː The Epidermolysis Bullosa (EB) is a rare congenital disease associated with blistering skin caused by minimal trauma. Because of its exposed position and its continued use in daily activities, the hand is constantly at risk of microtrauma and is therefore one of the organs most affected by the disease with highly disabling deformities that represents a challenging field in hand surgery practice.METHODSː The Authors present their experience in the microsurgical treatment of pseudosyndactylies comparing the classic dressing with vaseline gauze with our innovative “glove protocol” using a Dermal Regeneration Template. The endpoints analyzed were: healing times, hospital stay time, discomfort for the patient, free-recurrence interval, follow-up range and major complications.RESULTSː A total of 34 procedures were performed on 24 EB patients with hand deformities according to the proposed treatment strategy. We observed reduction of: hospital stay time, complete healing times, discomfort after dressings and average time to free recurrence.CONCLUSIONSː The microsurgical approach followed by our new protocol described in the study has been beneficial in providing consistent and successful long-term results for these patients.

scholarly journals New treatment protocol for pseudosyndactyly in epidermolysis bullosa hand using a dermal regeneration template glove.

2020 ◽  
Author(s):  
FEDELE LEMBO ◽  
DOMENICO PARISI ◽  
LIBERATO ROBERTO CECCHINO ◽  
FRANCESCO CIANCIO ◽  
ALESSANDRO INNOCENTI ◽  
...  
Keyword(s):  
Hospital Stay ◽  
Epidermolysis Bullosa ◽  
Treatment Protocol ◽  
Hand Surgery ◽  
Healing Time ◽  
Long Term Results ◽  
Minimal Trauma ◽  
Stay Time ◽  
Dermal Regeneration Template ◽  
Dermal Regeneration

Abstract BACKGROUNDː The Epidermolysis Bullosa (EB) is a rare genetic disease associated with blistering skin caused by minimal trauma. Because of its exposed position and its continued use in daily activities, the hand is constantly at risk of microtrauma and is therefore one of the organs most affected by the disease with highly disabling deformities that represents a challenging field in hand surgery practice.METHODSː The Authors present their experience in the microsurgical treatment of pseudosyndactylies comparing the classic dressing with vaseline gauze with our innovative “glove protocol” using Integra® dermal regeneration template. The endpoints analyzed were: healing times, hospital stay time, discomfort for the patient, free-recurrence interval, follow-up range and major complications.RESULTSː A total of 34 procedures were performed on 24 EB patients with hand deformities. Compared with the dressing with vaseline gauze, microsurgery followed by application of dermal regeneration template gloves allowed a significant reduction of hospital stay, healing time, and dressing pain as well as an increased recurrence-free interval.CONCLUSIONSː The microsurgical approach followed by our new protocol described in the study has been beneficial in providing consistent and successful long-term results for these patients.

Silver-impregnated foam dressings over circumferentially involved curved surfaces in epidermolysis bullosa hand surgery

2020 ◽  
Vol 83 (2) ◽  
pp. e111-e112
Author(s):  
Aditya Yadav ◽  
Satyaswarup Tripathy ◽  
Rahul Mahajan
Keyword(s):  
Epidermolysis Bullosa ◽  
Hand Surgery ◽  
Curved Surfaces

The use of Biobrane for hand surgery in Epidermolysis bullosa

2010 ◽  
Vol 63 (8) ◽  
pp. 1305-1311 ◽  
Author(s):  
J. Jutkiewicz ◽  
B.H. Noszczyk ◽  
M. Wrobel
Keyword(s):  
Epidermolysis Bullosa ◽  
Hand Surgery

Hand Surgery for Dystrophic Epidermolysis Bullosa

2014 ◽  
Vol 34 (7) ◽  
pp. 710-714 ◽  
Author(s):  
Shai Luria ◽  
Saleh Radwan ◽  
Gershon Zinger ◽  
Sharon Eylon
Keyword(s):  
Epidermolysis Bullosa ◽  
Hand Surgery ◽  
Dystrophic Epidermolysis Bullosa

scholarly journals Release of pseudosyndactyly in recessive dystrophic epidermolysis bullosa using a dermal regeneration template glove – the Foggia experience.

2021 ◽  
Author(s):  
Fedele Lembo ◽  
Domenico Parisi ◽  
Liberato Roberto Cecchinov ◽  
Francesco Ciancio ◽  
Alessandro Innocenti ◽  
...  
Keyword(s):  
Hospital Stay ◽  
Epidermolysis Bullosa ◽  
Genetic Diseases ◽  
Hand Surgery ◽  
Healing Time ◽  
Long Term Results ◽  
Minimal Trauma ◽  
Stay Time ◽  
Dermal Regeneration Template ◽  
Dermal Regeneration

Abstract BACKGROUNDː Epidermolysis Bullosa (EB) comprises a heterogeneous group of rare genetic diseases associated with skin blistering caused by minimal trauma. A major and common EB subtype, recessive dystrophic EB (RDEB), is characterized by altered wound healing, inflammatory dysbalance and fibrotic changes associated with reduced to absent collagen VII. Because of its exposed position and its continued use in daily activities, the hand is constantly at risk of microtrauma and is therefore one of the organs most affected by the disease with highly disabling deformities that represent a challenging field in hand surgery practice. METHODSː The Authors present their experience in the microsurgical treatment of pseudosyndactylies comparing the classic dressing with vaseline gauze with an innovative “glove protocol” using Integra® dermal regeneration template. The endpoints analyzed were: healing times, hospital stay time, discomfort for the patient, free-recurrence interval, follow-up range and major complications. RESULTSː A total of 34 procedures were performed on 24 RDEB patients with hand deformities. Compared with the dressing with vaseline gauze, microsurgery followed by application of dermal regeneration template gloves allowed a significant reduction of hospital stay, healing time, and dressing pain as well as an increased recurrence-free interval. CONCLUSIONSː The microsurgical approach followed by our new protocol described in the study has been beneficial in providing consistent and successful long-term results for these patients. Key words : Epidermolysis Bullosa, Pseudosyndactyly, Hand, Dermal regeneration template.

scholarly journals Release of pseudosyndactyly in recessive dystrophic epidermolysis bullosa using a dermal regeneration template glove: the Foggia experience

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Fedele Lembo ◽  
Domenico Parisi ◽  
Liberato Roberto Cecchino ◽  
Francesco Ciancio ◽  
Alessandro Innocenti ◽  
...  
Keyword(s):  
Hospital Stay ◽  
Epidermolysis Bullosa ◽  
Genetic Diseases ◽  
Hand Surgery ◽  
Healing Time ◽  
Long Term Results ◽  
Minimal Trauma ◽  
Stay Time ◽  
Dermal Regeneration Template ◽  
Dermal Regeneration

Abstract Background Epidermolysis bullosa (EB) comprises a heterogeneous group of rare genetic diseases associated with skin blistering caused by minimal trauma. A major and common EB subtype, recessive dystrophic EB (RDEB), is characterized by altered wound healing, inflammatory dysbalance and fibrotic changes associated with reduced to absent collagen VII. Because of its exposed position and its continued use in daily activities, the hand is constantly at risk of microtrauma and is therefore one of the organs most affected by the disease with highly disabling deformities that represent a challenging field in hand surgery practice. Methods The authors present their experience in the microsurgical treatment of pseudosyndactylies comparing the classic dressing with vaseline gauze with an innovative “glove protocol” using Integra® dermal regeneration template. The endpoints analyzed were: healing times, hospital stay time, discomfort for the patient, free-recurrence interval, follow-up range and major complications. Results A total of 34 procedures were performed on 24 RDEB patients with hand deformities. Compared with the dressing with vaseline gauze, microsurgery followed by application of dermal regeneration template gloves allowed a significant reduction of hospital stay, healing time, and dressing pain as well as an increased recurrence-free interval. Conclusions The microsurgical approach followed by our new protocol described in the study has been beneficial in providing consistent and successful long-term results for these patients.

Structure, compositon and function of the dermal-epidermal junction: Relevance in cutaneous disease and diagnosis

1989 ◽  
Vol 47 ◽  
pp. 868-869
Author(s):  
K. A. Holbrook
Keyword(s):  
Basement Membrane ◽  
Epidermolysis Bullosa ◽  
Normal Skin ◽  
Lamina Densa ◽  
Inherited Disorders ◽  
Ultrastructural Studies ◽  
Type Iv ◽  
Type Vii Collagen ◽  
Attachment Structures ◽  
Matrix Components

The dermal-epidermal junction (DEJ), or basement membrane rone, is the boundary between the epithelial and mesenchymal compartments of the skin; epidermal and fibroblastic cells in these two regions collaborate to synthesire its components. Ultrastructural studies (TEM and SEM) have defined a series of planes or layers (basal epidermal, lamina lucida, lamina densa, sublamina densa) and the morphology and density of attachment structures (hemidesmosomes, anchoring filaments, anchoring fibrils and anchoring plaques) in this region of normal skin. Change in structure of the DEJ provides information about the history of the tissue; reduplication of the lamina densa, for example, indicates a site of cell detachment or migration, or remodelling that accompanies repair of focal damage. In normal skin the structure of the DEJ is stable; in pathologic conditions it can be compromised by the congenital absence of certain structures or antigens (e.g., in the inherited disorders, epidermolysis bullosa [EB]) or by enzymatic degradation (e.g., in tumor invasion). Dissolution of the DEJ can also occur normally during the formation of epidermal appendages (e.g., hair follicles) and as melanocytes and Langerhans cells migrate into the epidermis during development.Biochemical and immunohisto/cytochemical studies have identified more than 20 molecules at the DEJ. These include well known matrix molecules (e.g., types IV and V collagen, laminin and fibronectin) and skin-specific antigens. The latter have been identified by autoantibodies or specific polyclonal or monoclonal antibodies raised against the skin, cultured cells and other epithelia. Some of the molecules of the DEJ are are present in basement membrane zones of many epithelia and thus are considered ubiquitous components (type IV, V, laminin, fibronectin, nidogen, entactin, HSPG, LDA-1, CSP [3B3]). All of them (that have been investigated in developing skin) appear ontogenetically as early as human embryonic tissue can be obtained and their expression is typically normal in patients with EB. The known properties of many of these molecules (particularly the matrix components) suggest functions they might impart to the DEJ: support of an epithelium (type IV collagen), regulation of permeability (heparan sulfate proteoglycan) or facilitation of cell attachment (fibronectin) and movement (laminin). Another group of matrix components and antigens of the DEJ includes molecules that are skin-specific or characteristic of stratified squamous epithelia (type VII collagen=LH 7:2 antigen, bullous pemphigoid antigen, AA3, GB3, KF-1,19-DEJ-1, epidermolysis bullosa acquisita antigen [EBA], AF-1 and AF-2, cicatricial pemphigoid antigen [CPA]) . These molecules are expressed in the DEJ later in development than the first group of molecules, in conjunction with the morphologic appearance of the structure they represent. Their appearance is also coordinated with specific developmental events (e.g., epidermal stratification) and the expression of molecules of differentiation in the epidermis and dermis. One or more of them is typically absent or reduced in expression in the skin of patients with heritable disorders affecting this region. There is no apparent correlation between the location of molecules in the DEJ and the stability of their expression.

Early management of infants with epidermolysis bullosa

2001 ◽  
Vol 26 (2) ◽  
pp. 213-213
Author(s):  
Jacqueline Denyer
Keyword(s):  
Epidermolysis Bullosa ◽  
Early Management
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