Pregnancy and delivery in a patient with mutilating dystrophic epidermolysis bullosa (Hallopeau-Siemens type)
Pregnancy and delivery in a patient with mutilating dystrophic epidermolysis bullosa (Hallopeau-Siemens type)
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DOI：
10.1016/s0029-7844(97)81421-4
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发表时间：
1997-05-01
影响因子：
7.2
通讯作者：
Dudenhausen, JW
中科院分区：
文献类型：
--
作者：
Buscher, U;Wessel, J;Dudenhausen, JW
背景：致残性Hallopeau-Siemens型大疱性表皮松解症是一种罕见的遗传性皮肤病。那些受影响的人在轻微创伤后会起水泡，皮肤和粘膜上会有明显的疤痕。在受影响的妇女中怀孕是非常罕见的。病例：一名24岁的妇女，孕妇1，患有严重的Hallopeau-Siemens类型，在怀孕期间被密切监测。患者在足月自然分娩了一名健康的女性新生儿。会阴切开创面愈合无并发症。七个月后，她在第二次怀孕时回来，当时合并了轻微的贫血和可能的妊娠期糖尿病导致的羊水过多。同样，健康新生儿的阴道分娩是在足月进行的。我们没有观察到妊娠引起的皮肤病的恶化。结论：Hallopeau-Siemens类型的大疱性表皮松解型营养不良的妇女可能在仔细评估损害程度和彻底解释与怀孕和分娩相关的风险后决定生孩子。密切监测孕妇是很重要的。阴道分娩应作为首选。母乳喂养是困难的，但并不是禁忌。(C)1997年，由美国妇产科医师学会颁发。
Background: Epidermolysis bullosa dystrophica of the mutilating Hallopeau-Siemens type is a rare inherited skin disease. Those afflicted have blisters and pronounced scarring of skin and mucous membranes after minor trauma. Pregnancies are very rare in affected women.Case: A 24-year-old woman, gravida 1, with a severe form of the Hallopeau-Siemens type was monitored closely during pregnancy. The patient spontaneously delivered a healthy female neonate at term. Episiotomy wound healing was uncomplicated. Seven months later, she returned in her second pregnancy, which was complicated by mild anemia and polyhydramnios from possible gestational-onset diabetes mellitus. Again, vaginal delivery of a healthy neonate was performed at term. We did not observe pregnancy-induced exacerbations of the skin disease.Conclusion: Women with epidermolysis bullosa dystrophica of the Hallopeau-Siemens type may decide to have children after careful evaluation of the degree of impairment and a thorough explanation of the risks associated with pregnancy and delivery. Close monitoring of the pregnant patient is important. Vaginal delivery should be the first choice. Breast-feeding is difficult, but not contraindicated. (C) 1997 by The American College of Obstetricians and Gynecologists.