Abrams Melissa L, Smidt Aimee, Benjamin Latanya, Chen Mei, Woodley David, Mancini Anthony J
Department of Dermatology, Northwestern University’s Feinberg School of Medicine, Chicago, Illinois, USA.
Arch Dermatol. 2011 Mar;147(3):337-41. doi: 10.1001/archdermatol.2010.317. Epub 2010 Nov 15.
Epidermolysis bullosa acquisita (EBA) is a rare, chronic, autoimmune bullous dermatosis that is caused by autoantibodies against the noncollagenous terminus of the α 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.
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.
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.
获得性大疱性表皮松解症（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，导致致密板层中的锚定原纤维减少。其典型表现为皮肤脆弱及创伤性水疱，尤其在四肢远端广泛出现，愈合后有粟丘疹、色素沉着和瘢痕形成，临床表现与营养不良型大疱性表皮松解症相似。发病通常在成年期，不过也有罕见的儿童病例。据我们所知，文献中尚未报道过涉及先天性EBA的新生儿病例。我们描述了一名因母体自身抗体被动转移而患短暂性EBA的新生儿。
一名2日龄女婴因自出生以来就存在的紧张性水疱和皮肤剥脱区域接受评估。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测定结果证实了新生儿EBA的诊断。患者经支持治疗后病情好转，且无需全身干预。
由母体IgG自身抗体经胎盘转移引起的自身免疫性新生儿大疱性皮肤病较为罕见。在患有寻常型天疱疮、落叶型天疱疮和妊娠类天疱疮的母亲所生的新生儿中曾有报道。据我们所知，先天性EBA此前尚未见报道。垂直获得的先天性自身免疫性水疱性疾病似乎具有自限性，通过支持治疗可缓解，同时推测母体自身抗体也会从新生儿循环中清除。
