{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,10]],"date-time":"2026-06-10T18:54:10Z","timestamp":1781117650092,"version":"3.54.1"},"reference-count":29,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,1,28]],"date-time":"2021-01-28T00:00:00Z","timestamp":1611792000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2021,1,28]],"date-time":"2021-01-28T00:00:00Z","timestamp":1611792000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Orphanet J Rare Dis"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      The authors present their experience in the microsurgical treatment of pseudosyndactylies comparing the classic dressing with vaseline gauze with an innovative \u201cglove protocol\u201d using Integra\n                      <jats:sup>\u00ae<\/jats:sup>\n                      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.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13023-021-01697-5","type":"journal-article","created":{"date-parts":[[2021,1,28]],"date-time":"2021-01-28T08:06:26Z","timestamp":1611821186000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Release of pseudosyndactyly in recessive dystrophic epidermolysis bullosa using a dermal regeneration template glove: the Foggia experience"],"prefix":"10.1186","volume":"16","author":[{"given":"Fedele","family":"Lembo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Domenico","family":"Parisi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liberato Roberto","family":"Cecchino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Ciancio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Innocenti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aurelio","family":"Portincasa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,1,28]]},"reference":[{"key":"1697_CR1","doi-asserted-by":"publisher","first-page":"614","DOI":"10.1111\/bjd.18921","volume":"183","author":"C Has","year":"2020","unstructured":"Has C, Bauer JW, Bodemer C, et al. Consensus reclassification of inherited epidermolysis bullosa and other disorders with skin fragility. Br J Dermatol. 2020;183:614\u201327.","journal-title":"Br J Dermatol"},{"key":"1697_CR2","doi-asserted-by":"publisher","first-page":"1231","DOI":"10.1001\/jamadermatol.2016.2473","volume":"152","author":"JD Fine","year":"2016","unstructured":"Fine JD. Epidemiology of inherited epidermolysis bullosa based on incidence and prevalence estimates from the National Epidermolysis Bullosa Registry. JAMA Dermatol. 2016;152:1231\u20138.","journal-title":"JAMA Dermatol"},{"key":"1697_CR3","doi-asserted-by":"publisher","first-page":"574","DOI":"10.1111\/bjd.18128","volume":"182","author":"C Has","year":"2020","unstructured":"Has C, Liu L, Bolling MC, et al. Clinical practice guidelines for laboratory diagnosis of epidermolysis bullosa. Br J Dermatol. 2020;182:574\u201392.","journal-title":"Br J Dermatol"},{"key":"1697_CR4","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/s40257-013-0059-z","volume":"15","author":"CK Hsu","year":"2014","unstructured":"Hsu CK, Wang SP, Lee JY, McGrath JA. Treatment of hereditary epidermolysis bullosa: updates and future prospects. Am J Clin Dermatol. 2014;15:1\u20136.","journal-title":"Am J Clin Dermatol"},{"key":"1697_CR5","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1016\/J.JHSB.2004.07.006","volume":"30","author":"JD Fine","year":"2005","unstructured":"Fine JD, Johnson L, Weiner M, et al. Pseudosyndactyly and musculoskeletal contractures in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry, 1986\u20132002. J Hand Surg Br. 2005;30:14\u201322.","journal-title":"J Hand Surg Br"},{"key":"1697_CR6","doi-asserted-by":"publisher","first-page":"5707","DOI":"10.3390\/ijms20225707","volume":"20","author":"AG Condorelli","year":"2019","unstructured":"Condorelli AG, Dellambra E, Logli E, Zambruno G, Castiglia D. Epidermolysis bullosa-associated squamous cell carcinoma: from pathogenesis to therapeutic perspectives. Int J Mol Sci. 2019;20:5707.","journal-title":"Int J Mol Sci"},{"key":"1697_CR7","doi-asserted-by":"publisher","first-page":"80","DOI":"10.1080\/2000656X.2017.1338183","volume":"52","author":"S Tuncer","year":"2018","unstructured":"Tuncer S, Sezgin B, Kaya B, Ayhan S, Latifoglu O. An algorithmic approach for the management of hand deformities in dystrophic epidermolysis bullosa. J Plast Surg Hand Surg. 2018;52:80\u20136.","journal-title":"J Plast Surg Hand Surg"},{"key":"1697_CR8","doi-asserted-by":"publisher","first-page":"1421","DOI":"10.1016\/j.bjps.2008.10.008","volume":"61","author":"A Burd","year":"2008","unstructured":"Burd A. RDEB: regeneration is not enough. J Plast Reconstr Aesthet Surg. 2008;61:1421\u20132.","journal-title":"J Plast Reconstr Aesthet Surg"},{"key":"1697_CR9","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1016\/0007-1226(92)90207-E","volume":"45","author":"PJ Terrill","year":"1992","unstructured":"Terrill PJ, Mayou BJ, Pemberton J. Experience in the surgical management of the hand in dystrophic epidermolysis bullosa. Br J Plast Surg. 1992;45:435\u201342.","journal-title":"Br J Plast Surg"},{"key":"1697_CR10","doi-asserted-by":"publisher","first-page":"76","DOI":"10.1186\/1750-1172-9-76","volume":"9","author":"M El Hachem","year":"2014","unstructured":"El Hachem M, Zambruno G, Bourdon-Lanoy E, et al. Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa. Orphanet J Rare Dis. 2014;9:76.","journal-title":"Orphanet J Rare Dis"},{"key":"1697_CR11","doi-asserted-by":"publisher","first-page":"710","DOI":"10.1097\/BPO.0000000000000152","volume":"34","author":"S Luria","year":"2014","unstructured":"Luria S, Radwan S, Zinger G, Eylon S. Hand surgery for dystrophic epidermolysis bullosa. J Pediatr Orthop. 2014;34:710\u20134.","journal-title":"J Pediatr Orthop"},{"key":"1697_CR12","doi-asserted-by":"crossref","first-page":"e780","DOI":"10.3928\/01477447-20110922-37","volume":"34","author":"F Tian","year":"2011","unstructured":"Tian F, Li B, Tian L-J. Treatment of severe hand deformities caused by epidermolysis bullosa. Orthopedics. 2011;34:e780\u20133.","journal-title":"Orthopedics"},{"key":"1697_CR13","first-page":"91","volume":"16","author":"GL Campiglio","year":"1997","unstructured":"Campiglio GL, Pajardi G, Rafanelli G. A new protocol for the treatment of hand deformities in recessive dystrophic epidermolysis bullosa (13 cases). Ann Chir Main Memb Super. 1997;16:91\u2013101.","journal-title":"Ann Chir Main Memb Super"},{"key":"1697_CR14","doi-asserted-by":"crossref","unstructured":"Formsma S a., Maathuis CBG, Robinson PH, Jonkman MF. Postoperative Hand Treatment in Children with Recessive Dystrophic Epidermolysis Bullosa. J Hand Ther. 2008;21:80\u201385","DOI":"10.1197\/j.jht.2007.10.001"},{"key":"1697_CR15","doi-asserted-by":"publisher","first-page":"254","DOI":"10.1097\/00000637-199509000-00006","volume":"35","author":"AO Ciccarelli","year":"1995","unstructured":"Ciccarelli AO, Rothaus KO, Carter DM, et al. Plastic and reconstructive surgery in epidermolysis bullosa: clinical experience with 110 procedures in 25 patients. Ann Plast Surg. 1995;35:254\u201361.","journal-title":"Ann Plast Surg"},{"key":"1697_CR16","doi-asserted-by":"publisher","first-page":"165","DOI":"10.2340\/00015555-0776","volume":"90","author":"K Natsuga","year":"2010","unstructured":"Natsuga K, Sawamura D, Goto M, Homma E, Goto-Ohguchi Y, Aoyagi S, Akiyama M, Kuroyanagi Y, Shimizu H. Response of intractable skin ulcers in recessive dystrophic epidermolysis bullosa patients to an allogeneic cultured dermal substitute. Acta Derm Venereol. 2010;90:165\u20139.","journal-title":"Acta Derm Venereol"},{"key":"1697_CR17","doi-asserted-by":"publisher","first-page":"1305","DOI":"10.1016\/j.bjps.2009.06.038","volume":"63","author":"J Jutkiewicz","year":"2010","unstructured":"Jutkiewicz J, Noszczyk BH, Wrobel M. The use of Biobrane for hand surgery in Epidermolysis bullosa. J Plast Reconstr Aesthet Surg. 2010;63:1305\u201311.","journal-title":"J Plast Reconstr Aesthet Surg"},{"key":"1697_CR18","doi-asserted-by":"publisher","first-page":"472","DOI":"10.1111\/iwj.12226","volume":"11","author":"E Sari","year":"2014","unstructured":"Sari E, Eryilmaz T, Tetik G, Ozakpinar HR, Eker E. Suprathel(\u00ae) -assisted surgical treatment of the hand in a dystrophic epidermolysis bullosa patient. Int Wound J. 2014;11:472\u20135.","journal-title":"Int Wound J"},{"key":"1697_CR19","doi-asserted-by":"publisher","first-page":"584","DOI":"10.1097\/01.PRS.0000070730.95956.01","volume":"112","author":"DP Fivenson","year":"2003","unstructured":"Fivenson DP, Scherschun L, Cohen LV. Apligraf in the treatment of severe mitten deformity associated with recessive dystrophic epidermolysis bullosa. Plast Reconstr Surg. 2003;112:584\u20138.","journal-title":"Plast Reconstr Surg"},{"key":"1697_CR20","doi-asserted-by":"publisher","first-page":"608","DOI":"10.1054\/bjps.1998.9997","volume":"51","author":"M Eisenberg","year":"1998","unstructured":"Eisenberg M, Llewelyn D. Surgical management of hands in children with recessive dystrophic epidermolysis bullosa: use of allogeneic composite cultured skin grafts. Br J Plast Surg. 1998;51:608\u201313.","journal-title":"Br J Plast Surg"},{"key":"1697_CR21","doi-asserted-by":"publisher","first-page":"379","DOI":"10.1097\/00000637-199910000-00006","volume":"43","author":"PD Witt","year":"1999","unstructured":"Witt PD, Cheng CJ, Mallory SB, et al. Surgical treatment of pseudosyndactyly of the hand in epidermolysis bullosa: histological analysis of an acellular allograft dermal matrix. Ann Plast Surg. 1999;43:379\u201385.","journal-title":"Ann Plast Surg"},{"key":"1697_CR22","doi-asserted-by":"publisher","first-page":"1225","DOI":"10.1001\/archderm.136.10.1225","volume":"136","author":"AF Falabella","year":"2000","unstructured":"Falabella AF, Valencia IC, Eaglstein WH, et al. Tissue-engineered skin (Apligraf) in the healing of patients with epidermolysis bullosa wounds. Arch Dermatol. 2000;136:1225\u201330.","journal-title":"Arch Dermatol"},{"key":"1697_CR23","doi-asserted-by":"publisher","first-page":"886","DOI":"10.1067\/mjd.2003.502","volume":"48","author":"DP Fivenson","year":"2003","unstructured":"Fivenson DP, Scherschun L, Choucair M, et al. Graftskin therapy in epidermolysis bullosa. J Am Acad Dermatol. 2003;48:886\u201392.","journal-title":"J Am Acad Dermatol"},{"key":"1697_CR24","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1001\/archderm.135.5.503","volume":"135","author":"PD Witt","year":"1999","unstructured":"Witt PD, Cohen DT, Mallory SB. Use of a permanent acellular dermal allograft in recessive dystrophic epidermolysis bullosa involving the hands. Arch Dermatol. 1999;135:503\u20136.","journal-title":"Arch Dermatol"},{"key":"1697_CR25","doi-asserted-by":"publisher","first-page":"380","DOI":"10.1159\/000246155","volume":"194","author":"P Verplancke","year":"1997","unstructured":"Verplancke P, Beele H, Monstrey S, et al. Treatment of dystrophic epidermolysis bullosa with autologous meshed split-thickness skin grafts and allogenic cultured keratinocytes. Dermatology. 1997;194:380\u20132.","journal-title":"Dermatology"},{"key":"1697_CR26","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1007\/s12593-013-0090-6","volume":"5","author":"\u00d6 \u00d6zkaya","year":"2016","unstructured":"\u00d6zkaya \u00d6, \u00d6rero\u011flu A, Akan M. The use of acellular dermal matrix in treatment of mitten hand in epidermolysis bullosa patients. J Hand Microsurg. 2016;5:46\u20137.","journal-title":"J Hand Microsurg"},{"key":"1697_CR27","first-page":"403","volume":"6","author":"F Lembo","year":"2012","unstructured":"Lembo F, Parisi D, Annacontini L, et al. Versatility of the dermal regeneration template (Integra\u00ae) engineered tissue in the reconstruction of different districts of the body. J Tissue Eng Regen Med. 2012;6:403.","journal-title":"J Tissue Eng Regen Med"},{"key":"1697_CR28","doi-asserted-by":"publisher","first-page":"1006","DOI":"10.1016\/j.ejso.2012.07.096","volume":"38","author":"F Lembo","year":"2012","unstructured":"Lembo F, Parisi D, Annacontini L, et al. Head reconstruction: 5 years experience with the Integra\u00ae dermal regeneration template. Eur J Surg Oncol. 2012;38:1006\u20137.","journal-title":"Eur J Surg Oncol"},{"key":"1697_CR29","doi-asserted-by":"publisher","first-page":"1109","DOI":"10.1111\/j.1460-9592.2008.02594.x","volume":"18","author":"S Meola","year":"2008","unstructured":"Meola S. Epydermolysis bullosa: a new technique for mask ventilation. Paediatr Anaesth. 2008;18:1109\u201311.","journal-title":"Paediatr Anaesth"}],"container-title":["Orphanet Journal of Rare Diseases"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-021-01697-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s13023-021-01697-5\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-021-01697-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,4,22]],"date-time":"2021-04-22T15:19:12Z","timestamp":1619104752000},"score":1,"resource":{"primary":{"URL":"https:\/\/ojrd.biomedcentral.com\/articles\/10.1186\/s13023-021-01697-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1,28]]},"references-count":29,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["1697"],"URL":"https:\/\/doi.org\/10.1186\/s13023-021-01697-5","relation":{"has-preprint":[{"id-type":"doi","id":"10.21203\/rs.3.rs-19651\/v3","asserted-by":"object"},{"id-type":"doi","id":"10.21203\/rs.3.rs-19651\/v1","asserted-by":"object"},{"id-type":"doi","id":"10.21203\/rs.3.rs-19651\/v2","asserted-by":"object"}]},"ISSN":["1750-1172"],"issn-type":[{"value":"1750-1172","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,1,28]]},"assertion":[{"value":"26 March 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 January 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"28 January 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"All subjects (or their parents) participating in the study gave written informed consent.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"All subjects (or their parents) participating in the study gave written informed consent.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"52"}}