表皮松解症并发症:高质量证据表明,表皮松解症与一系列累及手足、上呼吸道、外眼、肾、牙齿和口腔、软组织和GI呼吸道的并发症有关,增加了生长迟缓、癌症和过早死亡的风险。[35]Fine JD, Johnson LB, Weiner M, et al. Pseudosyndactyly and musculoskeletal contractures in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry, 1986-2002. J Hand Surg (Br). 2005;30:14-22.http://www.ncbi.nlm.nih.gov/pubmed/15620486?tool=bestpractice.com[36]Wright JT, Fine JD, Johnson L. Hereditary epidermolysis bullosa: oral manifestations and dental management. Pediatr Dent. 1993;15:242-248.http://www.ncbi.nlm.nih.gov/pubmed/8247897?tool=bestpractice.com[37]Fine JD, Johnson LB, Weiner M, et al. Tracheolaryngeal complications of inherited epidermolysis bullosa. Laryngoscope. 2007;117:1652-1660.http://www.ncbi.nlm.nih.gov/pubmed/17762793?tool=bestpractice.com[46]Fine JD, Johnson LB, Weiner M, et al. Cause-specific risks of childhood death in inherited epidermolysis bullosa. J Pediatr. 2008;152:276-280.http://www.ncbi.nlm.nih.gov/pubmed/18206702?tool=bestpractice.com[47]Fine JD, Johnson LB, Weiner M, et al. Gastrointestinal complications of inherited epidermolysis bullosa: cumulative experience of the National Epidermolysis Bullosa Registry. J Pediatr Gastroenterol Nutr. 2008;46:147-158.http://www.ncbi.nlm.nih.gov/pubmed/18223373?tool=bestpractice.com[48]Fine JD, Johnson LB, Weiner M, et al. Inherited epidermolysis bullosa (EB) and the risk of life-threatening skin-derived cancers: the National EB Registry experience, 1986-2006. J Am Acad Dermatol. 2009;60:203-211.http://www.ncbi.nlm.nih.gov/pubmed/19026465?tool=bestpractice.com[49]Fine JD, Johnson LB, Weiner M, et al. Eye involvement in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry. Am J Ophthalmol. 2004;138:254-262.http://www.ncbi.nlm.nih.gov/pubmed/15289135?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
表皮松解症并发症:高质量证据表明,表皮松解症与一系列累及手足、上呼吸道、外眼、肾、牙齿和口腔、软组织和GI呼吸道的并发症有关,增加了生长迟缓、癌症和过早死亡的风险。[35]Fine JD, Johnson LB, Weiner M, et al. Pseudosyndactyly and musculoskeletal contractures in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry, 1986-2002. J Hand Surg (Br). 2005;30:14-22.http://www.ncbi.nlm.nih.gov/pubmed/15620486?tool=bestpractice.com[36]Wright JT, Fine JD, Johnson L. Hereditary epidermolysis bullosa: oral manifestations and dental management. Pediatr Dent. 1993;15:242-248.http://www.ncbi.nlm.nih.gov/pubmed/8247897?tool=bestpractice.com[37]Fine JD, Johnson LB, Weiner M, et al. Tracheolaryngeal complications of inherited epidermolysis bullosa. Laryngoscope. 2007;117:1652-1660.http://www.ncbi.nlm.nih.gov/pubmed/17762793?tool=bestpractice.com[46]Fine JD, Johnson LB, Weiner M, et al. Cause-specific risks of childhood death in inherited epidermolysis bullosa. J Pediatr. 2008;152:276-280.http://www.ncbi.nlm.nih.gov/pubmed/18206702?tool=bestpractice.com[47]Fine JD, Johnson LB, Weiner M, et al. Gastrointestinal complications of inherited epidermolysis bullosa: cumulative experience of the National Epidermolysis Bullosa Registry. J Pediatr Gastroenterol Nutr. 2008;46:147-158.http://www.ncbi.nlm.nih.gov/pubmed/18223373?tool=bestpractice.com[48]Fine JD, Johnson LB, Weiner M, et al. Inherited epidermolysis bullosa (EB) and the risk of life-threatening skin-derived cancers: the National EB Registry experience, 1986-2006. J Am Acad Dermatol. 2009;60:203-211.http://www.ncbi.nlm.nih.gov/pubmed/19026465?tool=bestpractice.com[49]Fine JD, Johnson LB, Weiner M, et al. Eye involvement in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry. Am J Ophthalmol. 2004;138:254-262.http://www.ncbi.nlm.nih.gov/pubmed/15289135?tool=bestpractice.com
大疱性表皮松解症与抑郁症:有高质量证据证明,严重大疱性表皮松解症会导致抑郁症的发生。[62]Fine JD, Johnson LB, Weiner M, et al. Impact of inherited epidermolysis bullosa on parental interpersonal relationships, marital status, and family size. Br J Dermatol. 2005;152:1009-1014.http://www.ncbi.nlm.nih.gov/pubmed/15888161?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
大疱性表皮松解症与抑郁症:有高质量证据证明,严重大疱性表皮松解症会导致抑郁症的发生。[62]Fine JD, Johnson LB, Weiner M, et al. Impact of inherited epidermolysis bullosa on parental interpersonal relationships, marital status, and family size. Br J Dermatol. 2005;152:1009-1014.http://www.ncbi.nlm.nih.gov/pubmed/15888161?tool=bestpractice.com
水疱形成:有中等质量证据证明,口服四环素可以有效地抑制水疱的发展。[42]Weiner M, Stein A, Cash S, et al. Tetracycline and epidermolysis bullosa simplex: a double-blind, placebo-controlled, crossover randomized clinical trial. Br J Dermatol. 2004;150:613-614.http://www.ncbi.nlm.nih.gov/pubmed/15030362?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
水疱形成:有中等质量证据证明,口服四环素可以有效地抑制水疱的发展。[42]Weiner M, Stein A, Cash S, et al. Tetracycline and epidermolysis bullosa simplex: a double-blind, placebo-controlled, crossover randomized clinical trial. Br J Dermatol. 2004;150:613-614.http://www.ncbi.nlm.nih.gov/pubmed/15030362?tool=bestpractice.com
水疱形成:有质量差的证据证明,掌跖外用三氯化铝作为收敛剂,可以减少单纯型表皮松解症患者继发于多汗症的水疱。[43]Younger IR, Priestley GC, Tidman MJ. Aluminum chloride hexahydrate and blistering in epidermolysis bullosa simplex. J Am Acad Dermatol. 1990;23:930-931.http://www.ncbi.nlm.nih.gov/pubmed/2254479?tool=bestpractice.com
系统评价或者受试者>200名的随机对照临床试验(RCT)。
水疱形成:有质量差的证据证明,掌跖外用三氯化铝作为收敛剂,可以减少单纯型表皮松解症患者继发于多汗症的水疱。[43]Younger IR, Priestley GC, Tidman MJ. Aluminum chloride hexahydrate and blistering in epidermolysis bullosa simplex. J Am Acad Dermatol. 1990;23:930-931.http://www.ncbi.nlm.nih.gov/pubmed/2254479?tool=bestpractice.com