Congenital Epidermolysis Bullosa Acquisita Vertical Transfer of Maternal Autoantibody From Mother to Infant
Congenital Epidermolysis Bullosa Acquisita Vertical Transfer of Maternal Autoantibody From Mother to Infant
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DOI：
10.1001/archdermatol.2010.317
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发表时间：
2011-03-01
影响因子：
--
通讯作者：
Mancini, Anthony J.
中科院分区：
文献类型：
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作者：
Abrams, Melissa L.;Smidt, Aimee;Mancini, Anthony J.
背景资料：获得性大疱性表皮病（EBA）是一种罕见的慢性自身免疫性大疱性皮肤病，由针对VII型胶原α链非胶原末端的自身抗体引起，导致致密层中锚定原纤维减少。典型表现为皮肤脆性和创伤引起的水疱，尤其是在四肢远端广泛，愈合时出现粟粒疹、色素沉着和瘢痕，与营养不良性大疱性表皮病的表现相似。该病通常在成年期发病，但儿童期发病的病例很少。据我们所知，涉及一个先天性EBA的新生儿的情况下，尚未在文献中报道。我们描述了一个新生儿短暂的EBA由于被动转移的母体自身antibodies.Observations：一个2天大的女孩进行了评估紧张的水泡和裸露的皮肤，自出生以来一直存在的领域。她的母亲被诊断为EBA。组织病理学分析，免疫荧光研究和酶联免疫吸附试验的结果证实了新生儿EBA的诊断。患者改善与支持性治疗，并没有需要系统的intervention.Conclusions：自身免疫性新生儿大疱性皮肤病引起的胎盘转移的母亲IgG自身抗体是罕见的。在患有寻常型天疱疮、落叶型天疱疮和妊娠期类天疱疮的母亲所生的新生儿中已有报告。据我们所知，先天性EBA以前没有报道。垂直获得性先天性自身免疫性水疱性疾病似乎是自限性的，并通过支持性治疗解决，同时假定母体自身抗体从新生儿循环中清除。
Background: Epidermolysis bullosa acquisita (EBA) is a rare, chronic, autoimmune bullous dermatosis that is caused by autoantibodies against the noncollagenous terminus of the alpha chain of type VII collagen, resulting in decreased anchoring fibrils in the lamina densa. It classically presents with skin fragility and trauma-induced blisters that are particularly extensive over the distal aspect of the extremities and that heal with milia, dyspigmentation, and scarring, similar in presentation to dystrophic epidermolysis bullosa. Disease onset is typically in adulthood, although rare cases of childhood disease occur. To our knowledge, a case involving a neonate with congenital EBA has not yet been reported in the literature. We describe a newborn with transient EBA due to the passive transfer of maternal autoantibodies.Observations: A 2-day-old girl was evaluated for tense blisters and areas of denuded skin that had been present since birth. Her mother carried the diagnosis of EBA. The results of histopathologic analysis, immunofluorescence studies, and enzyme-linked immunosorbent assay confirmed the diagnosis of neonatal EBA. The patient improved with supportive therapy and has not required systemic intervention.Conclusions: Autoimmune neonatal bullous skin disease caused by placental transfer of maternal IgG autoantibodies is rare. It has been reported in neonates born to mothers with pemphigus vulgaris, pemphigus foliaceus, and gestational pemphigoid. To our knowledge, congenital EBA has not been previously reported. Vertically acquired congenital autoimmune blistering disorders appear to be self-limited and resolve with supportive therapy, concomitant with the presumed clearance of maternal autoantibodies from the neonate's circulation.