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妊娠类天疱疮的围产期结局:三例报告及文献复习

Perinatal Outcome of Pemphigoid Gestationis: A Report of Three Cases and Review of the Literature.

作者信息

Inatomi Ayako, Katsura Daisuke, Tokoro Shinsuke, Tsuji Shunichiro, Murakami Takashi

机构信息

Department of Obstetrics and Gynecology, Shiga University of Medical Science, Otsu, JPN.

Department of Obstetrics and Gynecology, Shiga University of Medical Science, Ostu, JPN.

出版信息

Cureus. 2024 Sep 3;16(9):e68582. doi: 10.7759/cureus.68582. eCollection 2024 Sep.

DOI:10.7759/cureus.68582
PMID:39371863
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11449879/
Abstract

Pemphigoid gestationis (PG) is a rare autoimmune blistering disorder that typically manifests during the second or third trimester of pregnancy. It is characterized by intensely pruritic urticarial plaques and blister formation, driven by an autoimmune response against the BP180 protein in the basement membrane. In this report, three cases of PG are presented, each illustrating distinct clinical courses and management strategies. The first case involves a 32-year-old primigravida at 31 weeks of gestation who presented with abdominal blisters that were unresponsive to topical steroids. Oral prednisone at a dosage of 15 mg was initiated at 33 weeks, leading to the resolution of the rash by 37 weeks. She subsequently delivered vaginally at 40 weeks. The second case concerns a 37-year-old multigravida who developed blisters on her limbs and abdomen at 27 weeks, which improved with the application of topical steroids. Due to a history of a previous cesarean section, she delivered via elective cesarean section at 38 weeks. The third case involves a 35-year-old multigravida who experienced fetal growth restriction starting from 29 weeks. She developed a mild erythematous, pruritic rash, and blisters at 33 weeks and required an emergency cesarean section at 33 weeks due to non-reassuring fetal status. The diagnosis of PG was confirmed postpartum. These cases underscore the clinical variability and potential complications associated with PG. They also suggest that the severity of PG's cutaneous manifestations may not directly correlate with pregnancy outcomes. Early detection and individualized management are crucial to optimizing both maternal and neonatal outcomes.

摘要

妊娠类天疱疮(PG)是一种罕见的自身免疫性水疱性疾病,通常在妊娠中期或晚期出现。其特征为剧烈瘙痒的荨麻疹斑块和水疱形成,由针对基底膜中BP180蛋白的自身免疫反应驱动。在本报告中,介绍了3例PG病例,每例都说明了不同的临床病程和管理策略。第一例涉及一名32岁初产妇,妊娠31周时出现腹部水疱,局部使用类固醇治疗无效。33周时开始口服泼尼松,剂量为15毫克,到37周时皮疹消退。她随后在40周时经阴道分娩。第二例涉及一名37岁经产妇,27周时四肢和腹部出现水疱,局部使用类固醇后病情好转。由于既往有剖宫产史,她在38周时通过择期剖宫产分娩。第三例涉及一名35岁经产妇,从29周开始出现胎儿生长受限。她在33周时出现轻度红斑、瘙痒性皮疹和水疱,由于胎儿状况不佳,在33周时需要紧急剖宫产。产后确诊为PG。这些病例强调了与PG相关的临床变异性和潜在并发症。它们还表明,PG皮肤表现的严重程度可能与妊娠结局无直接关联。早期检测和个体化管理对于优化母婴结局至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/254bb0c95b21/cureus-0016-00000068582-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/11c820a31394/cureus-0016-00000068582-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/60fb9fc60b4e/cureus-0016-00000068582-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/b125a4f06886/cureus-0016-00000068582-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/e8bf6bb20888/cureus-0016-00000068582-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/254bb0c95b21/cureus-0016-00000068582-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/11c820a31394/cureus-0016-00000068582-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/60fb9fc60b4e/cureus-0016-00000068582-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/b125a4f06886/cureus-0016-00000068582-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/e8bf6bb20888/cureus-0016-00000068582-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c66a/11449879/254bb0c95b21/cureus-0016-00000068582-i05.jpg

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