Bart综合征3例
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摘要
报告3例Bart综合征。患儿为2女1男。出生后2例女童表现为单侧从膝部至足背皮肤缺损,男童表现为双侧从膝部至足背皮肤缺损。3例患儿均采用保守治疗,缺损皮肤恢复良好。随访至今,3例患儿皮肤缺损处表现为色素减退样瘢痕。该文对3例患者的临床资料进行总结并对相关文献进行了回顾。
Three cases of Bart syndrome are reported. The patients included two girls and one boy. Two girls presented with congenital localized absence of skin(CLAS) on the anterior and lateral surface of the right lower leg and the right dorsal foot at birth. The boy presented with bilateral and symmetrical congenital localized absence of skin on the anterior and lateral surface of both lower legs and dorsal feet at birth. All patients were given conservative treatments. The wounds completely healed with hypopigmented scars. Their clinical data were summarized, and related literatures were reviewed.
Three cases of Bart syndrome are reported. The patients included two girls and one boy. Two girls presented with congenital localized absence of skin(CLAS) on the anterior and lateral surface of the right lower leg and the right dorsal foot at birth. The boy presented with bilateral and symmetrical congenital localized absence of skin on the anterior and lateral surface of both lower legs and dorsal feet at birth. All patients were given conservative treatments. The wounds completely healed with hypopigmented scars. Their clinical data were summarized, and related literatures were reviewed.
引文
[1]Kulali F,Bas AY,Kale Y,et al.TypeⅥAplasia Cutis Congenita:Bart’s Syndrome[J].Case Rep Dermatol Med,2014,(2015):1-3.
[2]Silberstein E,Pagkalos VA,Landau D,et al.Aplasia cutis congenital:clinical management and a new classification system[J].Plast Reconstr Surg,2014,134(5):766e-774e.
[3]Aygun AD,Yilmaz E,Kurt AN,et al.Aplasia cutis congenita and epidemolysis bullosa:bart syndrome[J].Int J Dermatol,2010,49((3):343-345.
[4]Kuvat SV,Bozkurt M.Conservative treatment of a patient with epidermolysis bullosa presenting as bart syndrome:A case report[J].Case Rep Med,2010,2010(2):302345.
[5]Christiano AM,Bart BJ,Epstein EH Jr,et al.Genetic basis of Bart’s syndrome:A glycine substiution mutation in the typeⅧcollagen gene[J].J Invest Dermatol,1996,106(4):778-780.
[6]Rajpal A,Mishra R,Hajirnis K,et al.Bart’s syndrome[J].Indian J Dermatol,2008,53(2):88-90.
[7]Wakasugi S,Mizutari K,Ono T.Clinical phenotype of Bart’s syndrome seen in a family with dominant dystrophic epidermolysis bullosa[J].J Dermatol,1998,25(8):517-522.
[8]Duran-Mckinster C,Rivera-Franco A,Tamayo L,et al.Bart syndrome:the congenital localized absence of skin may follow the lines of blaschko.Report of six cases[J].Pediatr Dermatol,2000,17(3):179-182.
[9]陈晨,董仲陵.先天性皮肤缺损合并大疱性表皮松解症1例[J].中国民康医学,2012,24(15):1921-1922.
[10]廉佳,宫泽琨,李钦峰,等.Bart综合征[J].临床皮肤科杂志,2014,43(3):153-154.
[11]欧卉.磺胺嘧啶银脂质水胶体敷料治疗小面积皮肤缺损并伤口感染:生物相容性评价[J].中国组织工程研究,2015,19(21):3366-3370.
[2]Silberstein E,Pagkalos VA,Landau D,et al.Aplasia cutis congenital:clinical management and a new classification system[J].Plast Reconstr Surg,2014,134(5):766e-774e.
[3]Aygun AD,Yilmaz E,Kurt AN,et al.Aplasia cutis congenita and epidemolysis bullosa:bart syndrome[J].Int J Dermatol,2010,49((3):343-345.
[4]Kuvat SV,Bozkurt M.Conservative treatment of a patient with epidermolysis bullosa presenting as bart syndrome:A case report[J].Case Rep Med,2010,2010(2):302345.
[5]Christiano AM,Bart BJ,Epstein EH Jr,et al.Genetic basis of Bart’s syndrome:A glycine substiution mutation in the typeⅧcollagen gene[J].J Invest Dermatol,1996,106(4):778-780.
[6]Rajpal A,Mishra R,Hajirnis K,et al.Bart’s syndrome[J].Indian J Dermatol,2008,53(2):88-90.
[7]Wakasugi S,Mizutari K,Ono T.Clinical phenotype of Bart’s syndrome seen in a family with dominant dystrophic epidermolysis bullosa[J].J Dermatol,1998,25(8):517-522.
[8]Duran-Mckinster C,Rivera-Franco A,Tamayo L,et al.Bart syndrome:the congenital localized absence of skin may follow the lines of blaschko.Report of six cases[J].Pediatr Dermatol,2000,17(3):179-182.
[9]陈晨,董仲陵.先天性皮肤缺损合并大疱性表皮松解症1例[J].中国民康医学,2012,24(15):1921-1922.
[10]廉佳,宫泽琨,李钦峰,等.Bart综合征[J].临床皮肤科杂志,2014,43(3):153-154.
[11]欧卉.磺胺嘧啶银脂质水胶体敷料治疗小面积皮肤缺损并伤口感染:生物相容性评价[J].中国组织工程研究,2015,19(21):3366-3370.