A mechanobullous disease of the newborn. Bart's syndrome.
A mechanobullous disease of the newborn. Bart's syndrome.
复制标题
新生儿机械大疱性疾病。
DOI：
10.1001/archderm.1978.01640130045013
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发表时间：
1978
影响因子：
--
通讯作者：
D. Cram
中科院分区：
文献类型：
--
作者：
S. Z. Smith;D. Cram
一名患者患有一种独特的机械性延髓疾病(巴特氏综合征)。有特征性的先天性局部皮肤缺陷、创伤引起的侵蚀和指甲畸形。遗传模式似乎是常染色体显性遗传，但正如本报告所述，个别病例已被确认。在对其他机械性延髓疾病的回顾中，巴特氏综合征似乎呈现出独特的临床图景和病程，并具有良好的预后。渐进性自发改善强调早期识别和保守治疗的重要性。
A patient had a unique mechanobullous disease (Bart's syndrome). The characteristic congenital localized skin defects, trauma-induced erosions, and nail deformities are present. The inheritance pattern appears to be autosomal dominant but, as in this report, isolated cases have been recognized. In a review of the other mechanobullous diseases, Bart's syndrome appears to present a distinctive clinical picture and course and has a favorable prognosis. The progressive spontaneous improvement emphasizes the importance of early recognition and conservative treatment.