Literature DB >> 33509245

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

Fedele Lembo1, Domenico Parisi2, Liberato Roberto Cecchino2, Francesco Ciancio2, Alessandro Innocenti3, Aurelio Portincasa2.   

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.

Entities:  

Keywords:  Dermal regeneration template; Epidermolysis bullosa; Hand; Pseudosyndactyly

Mesh:

Year:  2021        PMID: 33509245      PMCID: PMC7845051          DOI: 10.1186/s13023-021-01697-5

Source DB:  PubMed          Journal:  Orphanet J Rare Dis        ISSN: 1750-1172            Impact factor:   4.123


  27 in total

1.  Use of a permanent acellular dermal allograft in recessive dystrophic epidermolysis bullosa involving the hands.

Authors:  P D Witt; D T Cohen; S B Mallory
Journal:  Arch Dermatol       Date:  1999-05

2.  Postoperative hand treatment in children with recessive dystrophic epidermolysis bullosa.

Authors:  S A Formsma; C B G Maathuis; P H Robinson; M F Jonkman
Journal:  J Hand Ther       Date:  2008 Jan-Mar       Impact factor: 1.950

3.  Epidermolysis bullosa: a new technique for mask ventilation.

Authors:  Salvatore Meola
Journal:  Paediatr Anaesth       Date:  2008-11       Impact factor: 2.556

4.  An algorithmic approach for the management of hand deformities in dystrophic epidermolysis bullosa.

Authors:  Serhan Tuncer; Billur Sezgin; Basar Kaya; Suhan Ayhan; Osman Latifoglu
Journal:  J Plast Surg Hand Surg       Date:  2017-07-02

5.  Surgical management of hands in children with recessive dystrophic epidermolysis bullosa: use of allogeneic composite cultured skin grafts.

Authors:  M Eisenberg; D Llewelyn
Journal:  Br J Plast Surg       Date:  1998-12

6.  Tissue-engineered skin (Apligraf) in the healing of patients with epidermolysis bullosa wounds.

Authors:  A F Falabella; I C Valencia; W H Eaglstein; L A Schachner
Journal:  Arch Dermatol       Date:  2000-10

7.  Pseudosyndactyly and musculoskeletal contractures in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry, 1986-2002.

Authors:  J-D Fine; L B Johnson; M Weiner; A Stein; S Cash; J Deleoz; D T Devries; C Suchindran
Journal:  J Hand Surg Br       Date:  2005-02

8.  Surgical treatment of pseudosyndactyly of the hand in epidermolysis bullosa: histological analysis of an acellular allograft dermal matrix.

Authors:  P D Witt; C J Cheng; S B Mallory; A C Lind
Journal:  Ann Plast Surg       Date:  1999-10       Impact factor: 1.539

9.  Suprathel(®) -assisted surgical treatment of the hand in a dystrophic epidermolysis bullosa patient.

Authors:  Elif Sari; Tolga Eryilmaz; Gulsum Tetik; Hulda R Ozakpinar; Esabil Eker
Journal:  Int Wound J       Date:  2014-02-11       Impact factor: 3.315

Review 10.  Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa.

Authors:  May El Hachem; Giovanna Zambruno; Eva Bourdon-Lanoy; Annalisa Ciasulli; Christiane Buisson; Smail Hadj-Rabia; Andrea Diociaiuti; Carolina F Gouveia; Angela Hernández-Martín; Raul de Lucas Laguna; Mateja Dolenc-Voljč; Gianluca Tadini; Guglielmo Salvatori; Cristiana De Ranieri; Stephanie Leclerc-Mercier; Christine Bodemer
Journal:  Orphanet J Rare Dis       Date:  2014-05-20       Impact factor: 4.123

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