Overview
Acquired epidermolysis bullosa (EB) encompasses a spectrum of conditions characterized by skin fragility leading to blister formation and subsequent scarring, often requiring multidisciplinary care due to its impact on quality of life and systemic complications. 123456789101620263139Diagnosis
Management
Special Populations
Key Recommendations
References
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Archives of dermatological research 2025. link 4 Hirt N, Manchon E, Chen Q, Delaroque C, Corneau A, Hemon P et al.. Systems immunology integrates the complex endotypes of recessive dystrophic epidermolysis bullosa. Nature communications 2025. link 5 Saad R, Duipmans J, Yerlett N, Plevey K, McCuaig C, Woolfe W et al.. Neonatal epidermolysis bullosa: a clinical practice guideline. The British journal of dermatology 2024. link 6 Abreu Molnar B, Levin L, Yun D, Morel K, Wiss K, Wieser J et al.. Inpatient management of epidermolysis bullosa: Consensus-based hands-on instructions for neonates and postneonates. Journal of the American Academy of Dermatology 2024. link 7 Snelson K, Downe A. Use of topical morphine gel in epidermolysis bullosa wounds-A series of case studies. International wound journal 2023. link 8 Box R, Bernardis C, Pleshkov A, Jessop N, Miller C, Skye J et al.. Hand surgery and hand therapy clinical practice guideline for epidermolysis bullosa. Orphanet journal of rare diseases 2022. link 9 Mittal BM, Goodnough CL, Bushell E, Turkmani-Bazzi S, Sheppard K. Anesthetic Management of Adults With Epidermolysis Bullosa. Anesthesia and analgesia 2022. link 10 Chernyshov PV, Marron SE, Tomas-Aragones L, Pustišek N, Gedeon I, Suru A et al.. Initial validation of the epidermolysis bullosa-specific module of the Infants and Toddlers Dermatology Quality of Life questionnaire. Dermatologic therapy 2020. link 11 Mazurova S, Tesarova M, Zeman J, Stranecky V, Hansikova H, Baxova A et al.. Fatal neonatal nephrocutaneous syndrome in 18 Roma children with EGFR deficiency. The Journal of dermatology 2020. link 12 Han YM, Lee N, Byun SY, Cheon SJ, Ko HC. Bart's Syndrome with Novel Frameshift Mutations in the COL7A1 Gene. Fetal and pediatric pathology 2019. link 13 van den Heuvel I, Gottschalk A, Langer M, Hahnenkamp K, Ellger B. Feasibility, efficacy, and safety of ultrasound-guided axillary plexus blockade in pediatric patients with epidermolysis bullosa dystrophica. Paediatric anaesthesia 2016. link 14 Mellerio JE, Robertson SJ, Bernardis C, Diem A, Fine JD, George R et al.. Management of cutaneous squamous cell carcinoma in patients with epidermolysis bullosa: best clinical practice guidelines. The British journal of dermatology 2016. link 15 Fortuna G, Aria M, Cepeda-Valdes R, Salas-Alanís JC. Evaluation of internal consistency of the epidermolysis bullosa oropharyngeal severity score (EBOS). Acta odontologica Scandinavica 2015. link 16 Goldschneider KR, Good J, Harrop E, Liossi C, Lynch-Jordan A, Martinez AE et al.. Pain care for patients with epidermolysis bullosa: best care practice guidelines. BMC medicine 2014. link 17 Terra JB, Meijer JM, Jonkman MF, Diercks GF. The n- vs. u-serration is a learnable criterion to differentiate pemphigoid from epidermolysis bullosa acquisita in direct immunofluorescence serration pattern analysis. The British journal of dermatology 2013. link 18 Fortuna G, Lozada-Nur F, Pollio A, Aria M, Cepeda-Valdes R, Marinkovich MP et al.. Patterns of oral mucosa lesions in patients with epidermolysis bullosa: comparison and agreement between oral medicine and dermatology. Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology 2013. link 19 Van Den Heuvel I, Boschin M, Langer M, Frosch M, Gottschalk A, Ellger B et al.. Anesthetic management in pediatric patients with epidermolysis bullosa: a single center experience. Minerva anestesiologica 2013. link 20 Tolar J, Wagner JE. Management of severe epidermolysis bullosa by haematopoietic transplant: principles, perspectives and pitfalls. Experimental dermatology 2012. link 21 Sproule TJ, Roopenian DC, Sundberg JP. A direct method to determine the strength of the dermal-epidermal junction in a mouse model for epidermolysis bullosa. Experimental dermatology 2012. link 22 Charruyer A, Ghadially R. What's new in dermatology: epidermal stem cells. Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia 2011. link 23 Martinez AE. Tests to monitor in patients with severe types of epidermolysis bullosa. Dermatologic clinics 2010. link 24 Duipmans JC, Jonkman MF. Epidermolysis bullosa in the Netherlands. Dermatologic clinics 2010. link 25 Swinburne C. Handle with care. Nursing standard (Royal College of Nursing (Great Britain) : 1987) 2010. link 26 Uitto J, McGrath JA, Rodeck U, Bruckner-Tuderman L, Robinson EC. Progress in epidermolysis bullosa research: toward treatment and cure. The Journal of investigative dermatology 2010. link 27 Duipmans JC, Jonkman MF. Interdisciplinary management of epidermolysis bullosa in the public setting: the Netherlands as a model of care. Dermatologic clinics 2010. link 28 Akar M, Erdeve O, Gokmen T, Oguz SS, Dilmen U, Sirvan L. Visceral bullae: A new finding in Bart's syndrome. Fetal and pediatric pathology 2010. link 29 Happle R. The group of epidermal nevus syndromes Part I. Well defined phenotypes. Journal of the American Academy of Dermatology 2010. link 30 Gottschalk A, Venherm S, Vowinkel T, Tübergen D, Frosch M, Hahnenkamp K. Anesthesia for balloon dilatation of esophageal strictures in children with epidermolysis bullosa dystrophica: from intubation to sedation. Current opinion in anaesthesiology 2010. link 31 Goldschneider KR, Lucky AW. Pain management in epidermolysis bullosa. Dermatologic clinics 2010. link 32 Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II. Other organs. Journal of the American Academy of Dermatology 2009. link 33 Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. Journal of the American Academy of Dermatology 2009. link 34 Saroyan JM, Tresgallo ME, Farkouh C, Morel KD, Schechter WS. The use of oral ketamine for analgesia with dressing change in an infant with epidermolysis bullosa: report of a case. Pediatric dermatology 2009. link 35 Hore I, Bajaj Y, Denyer J, Martinez AE, Mellerio JE, Bibas T et al.. The management of general and disease specific ENT problems in children with Epidermolysis Bullosa--a retrospective case note review. International journal of pediatric otorhinolaryngology 2007. link 36 Lin YC, Golianu B. Anesthesia and pain management for pediatric patients with dystrophic epidermolysis bullosa. Journal of clinical anesthesia 2006. link 37 Bart BJ, Lussky RC. Bart syndrome with associated anomalies. American journal of perinatology 2005. link 38 Crowley KL, Shevchenko YO. Anesthetic management of a difficult airway in a patient with epidermolysis bullosa: a case report. AANA journal 2004. link 39 Ingen-Housz-Oro S, Blanchet-Bardon C, Vrillat M, Dubertret L. Vitamin and trace metal levels in recessive dystrophic epidermolysis bullosa. Journal of the European Academy of Dermatology and Venereology : JEADV 2004. link 40 Dank JP, Kim S, Parisi MA, Brown T, Smith LT, Waldhausen J et al.. Outcome after surgical repair of junctional epidermolysis bullosa-pyloric atresia syndrome: a report of 3 cases and review of the literature. Archives of dermatology 1999. link 41 Ha D, Idikio H, Krol A, Lin AN. Junctional epidermolysis bullosa with pyloric atresia: A case with favourable outcome. Journal of cutaneous medicine and surgery 1998. link 42 Wakasugi S, Mizutari K, Ono T. Clinical phenotype of Bart's syndrome seen in a family with dominant dystrophic epidermolysis bullosa. The Journal of dermatology 1998. link 43 Pulkkinen L, Rouan F, Bruckner-Tuderman L, Wallerstein R, Garzon M, Brown T et al.. Novel ITGB4 mutations in lethal and nonlethal variants of epidermolysis bullosa with pyloric atresia: missense versus nonsense. American journal of human genetics 1998. link 44 Shaw DW, Fine JD, Piacquadio DJ, Greenberg MJ, Wang-Rodriguez J, Eichenfield LF. Gastric outlet obstruction and epidermolysis bullosa. Journal of the American Academy of Dermatology 1997. link80404-5) 45 Takizawa Y, Shimizu H, Nishikawa T, Hatta N, Pulkkinen L, Uitto J. Novel ITGB4 mutations in a patient with junctional epidermolysis bullosa-pyloric atresia syndrome and altered basement membrane zone immunofluorescence for the alpha6beta4 integrin. The Journal of investigative dermatology 1997. link 46 Valari MD, Phillips RJ, Lake BD, Harper JI. Junctional epidermolysis bullosa and pyloric atresia: a distinct entity. Clinical and pathological studies in five patients. 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Cultured epithelia from junctional epidermolysis bullosa letalis keratinocytes express the main phenotypic characteristics of the disease. The British journal of dermatology 1990. link 52 Fine JD, Tamura T, Johnson L. Blood vitamin and trace metal levels in epidermolysis bullosa. Archives of dermatology 1989. link 53 Bruckner-Tuderman L, Schnyder UW, Winterhalter KH, Bruckner P. Tissue form of type VII collagen from human skin and dermal fibroblasts in culture. European journal of biochemistry 1987. link 54 Haber RM, Hanna W, Ramsay CA, Boxall LB. Hereditary epidermolysis bullosa. Journal of the American Academy of Dermatology 1985. link70168-5) 55 Bull MJ, Norins AL, Weaver DD, Weber T, Mitchell M. Epidermolysis bullosa--pyloric atresia. An autosomal recessive syndrome. American journal of diseases of children (1960) 1983. link 56 Löfberg L, Anton-Lamprecht I, Michaëlsson G, Gustavii B. Prenatal exclusion of Herlitz syndrome by electron microscopy of fetal skin biopsies obtained at fetoscopy. Acta dermato-venereologica 1983. link 57 Skoven I, Drzewiecki KT. Congenital localized skin defect and epidermolysis bullosa hereditaria letalis. Acta dermato-venereologica 1979. link