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大疱性表皮松解症手部畸形的手术治疗：初步经验与技术

Surgical Management of Hand Deformity in Epidermolysis Bullosa: Initial Experience and Technique.

作者信息

Anstadt Erin E, Turer David M, Spiess Alexander M, Manders Ernest K

机构信息

Department of Plastic Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pa.

出版信息

Plast Reconstr Surg Glob Open. 2020 Mar 11;8(3):e2666. doi: 10.1097/GOX.0000000000002666. eCollection 2020 Mar.

DOI:10.1097/GOX.0000000000002666
PMID:32537331
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7253274/
Abstract

Epidermolysis bullosa describes a rare group of genetic mucocutaneous disorders characterized by excessive epithelial fragility resulting in mechanically induced blistering and abnormal wound healing. Its prevalence and incidence are 8.2 and 19.6 per 1,000,000 live births, respectively. Gene therapy, protein replacement, and cell therapy strategies have been investigated, but there is currently no cure..

摘要

大疱性表皮松解症描述了一组罕见的遗传性皮肤黏膜疾病，其特征是上皮细胞过度脆弱，导致机械性诱导水疱形成和伤口愈合异常。其患病率和发病率分别为每100万活产儿8.2例和19.6例。基因治疗、蛋白质替代和细胞治疗策略已被研究，但目前尚无治愈方法。

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Surgical Management of Hand Deformity in Epidermolysis Bullosa: Initial Experience and Technique.大疱性表皮松解症手部畸形的手术治疗：初步经验与技术
Plast Reconstr Surg Glob Open. 2020 Mar 11;8(3):e2666. doi: 10.1097/GOX.0000000000002666. eCollection 2020 Mar.
2
Self-initiated use of topical cannabidiol oil for epidermolysis bullosa.表皮松解性大疱病患者自行使用局部大麻二酚油剂的情况。
Pediatr Dermatol. 2018 Jul;35(4):e224-e227. doi: 10.1111/pde.13545. Epub 2018 May 22.
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Nutritional support for children with epidermolysis bullosa.大疱性表皮松解症患儿的营养支持
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Epidermolysis bullosa: new and emerging trends.大疱性表皮松解症：新趋势与新兴趋势
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Inside out: regenerative medicine for recessive dystrophic epidermolysis bullosa.从内到外：隐性营养不良型大疱性表皮松解症的再生医学。
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Cleft lip and cleft palate closure in 13 month-old female with Epidermolysis Bullosa.
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Functional therapies for cutaneous wound repair in epidermolysis bullosa.表皮松解性水疱症的皮肤伤口修复的功能疗法。
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Case report: rapidly healing epidermolysis bullosa wound after ablative fractional resurfacing.病例报告：消融性微剥脱术后快速愈合的大疱性表皮松解症伤口。
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Thymosin β4: potential to treat epidermolysis bullosa and other severe dermal injuries.胸腺素β4：治疗大疱性表皮松解症及其他严重皮肤损伤的潜力。
Eur J Dermatol. 2019 Oct 1;29(5):459-467. doi: 10.1684/ejd.2019.3642.
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Anaesthesia for children with epidermolysis bullosa: a review of 20 years' experience.大疱性表皮松解症患儿的麻醉：20年经验回顾
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引用本文的文献

1
"It's like nursing a butterfly-so delicate, difficult, and unpredictable" - challenges of nurses in caring for patients with epidermolysis bullosa: a qualitative study.“这就像照料一只蝴蝶——如此 delicate、困难且 unpredictable”——护士护理大疱性表皮松解症患者面临的挑战：一项定性研究。 （注：原文中delicate、unpredictable未给出准确中文释义，可根据语境灵活翻译，比如“ delicate”可译为“ delicate”可译为“精细的”“脆弱的”等，“unpredictable”可译为“不可预测的”“变幻莫测的”等 ）
Orphanet J Rare Dis. 2025 Jul 23;20(1):371. doi: 10.1186/s13023-025-03925-8.

本文引用的文献

1
Hereditary epidermolysis bullosa.遗传性大疱性表皮松解症
J Dtsch Dermatol Ges. 2015 Nov;13(11):1125-33. doi: 10.1111/ddg.12774.
2
Treatment of hereditary epidermolysis bullosa: updates and future prospects.遗传性大疱性表皮松解症的治疗：最新进展与未来展望
Am J Clin Dermatol. 2014 Feb;15(1):1-6. doi: 10.1007/s40257-013-0059-z.
3
Treatment of severe hand deformities caused by epidermolysis bullosa.
Orthopedics. 2011 Nov 9;34(11):e780-3. doi: 10.3928/01477447-20110922-37.
4
The use of Biobrane for hand surgery in Epidermolysis bullosa.生物敷贴在大疱性表皮松解症手部手术中的应用。
J Plast Reconstr Aesthet Surg. 2010 Aug;63(8):1305-11. doi: 10.1016/j.bjps.2009.06.038. Epub 2009 Jul 23.
5
Pseudosyndactyly and musculoskeletal contractures in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry, 1986-2002.遗传性大疱性表皮松解症中的假性并指畸形和肌肉骨骼挛缩：1986 - 2002年国家大疱性表皮松解症登记处的经验
J Hand Surg Br. 2005 Feb;30(1):14-22. doi: 10.1016/j.jhsb.2004.07.006.
6
Apligraf in the treatment of severe mitten deformity associated with recessive dystrophic epidermolysis bullosa.Apligraf用于治疗与隐性营养不良性大疱性表皮松解症相关的严重连指手套样畸形。
Plast Reconstr Surg. 2003 Aug;112(2):584-8. doi: 10.1097/01.PRS.0000070730.95956.01.
7
Surgical treatment and postoperative splinting of recessive dystrophic epidermolysis bullosa.隐性营养不良型大疱性表皮松解症的手术治疗及术后夹板固定
J Hand Surg Am. 1996 Sep;21(5):888-97. doi: 10.1016/S0363-5023(96)80210-3.
8
Plastic and reconstructive surgery in epidermolysis bullosa: clinical experience with 110 procedures in 25 patients.大疱性表皮松解症的整形与重建手术：25例患者110例手术的临床经验
Ann Plast Surg. 1995 Sep;35(3):254-61. doi: 10.1097/00000637-199509000-00006.