Neonatal Linear IgA Bullous Dermatosis Mediated by Breast Milk-Borne Maternal IgA
Neonatal Linear IgA Bullous Dermatosis Mediated by Breast Milk-Borne Maternal IgA
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DOI：
10.1001/jamadermatol.2021.2392
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发表时间：
2021-07-14
期刊：
影响因子：
10.9
通讯作者：
Amagai, Masayuki
中科院分区：
文献类型：
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作者：
Egami, Shohei;Suzuki, Chihiro;Amagai, Masayuki
新生儿线性免疫球蛋白A（伊加）大疱性皮肤病（LABD）是一种罕见的疾病，当与呼吸衰竭相关时可能是致命的。以往报道的所有新生儿LABD病例均发生在健康无症状母亲所生的新生儿中，致病性伊加来源不明。和参与者本病例研究分析了一个单一的乳房的实验室检查结果-喂养的患有新生儿LABD的新生儿男性和他的健康无症状母亲，他们被收住东京庆应义塾大学医院。健康新生儿在出生后第4天出现危及生命的水泡和皮肤粘膜糜烂。检测血清、皮肤及母乳中伊加自身抗体。主要结果与指标结果新生儿皮肤的组织学评价显示表皮下水疱伴中性粒细胞浸润，免疫荧光检测显示沿着基底膜区（BMZ）线性伊加沉积，从而诊断为新生儿LABD。用1-mol/L氯化钠治疗后使用正常人皮肤的间接免疫荧光显示患者具有与BMZ的真皮侧结合的循环伊加。免疫组化染色证明分泌型伊加沉积在新生儿皮肤中，通过展示J链的存在-在其他LABD病例中未观察到-表明产生水泡的自身抗体来自母亲母乳。虽然在母亲的血清中没有检测到针对皮肤的循环伊加，但母乳中含有与BMZ的真皮侧反应的伊加。没有新的水泡形成后，观察到停止母乳喂养。结论和相关性-本案例研究的结果表明，被动转移的病原性伊加的新生儿从一个无症状的母亲通过母乳。在以前的报告中，没有来自LABD新生儿的无症状母亲的血清具有与皮肤成分结合的伊加自身抗体;然而，在这种情况下，我们发现母亲的母乳含有与新生儿LABD相关的伊加自身抗体。在新生儿LABD中，应检查母亲母乳中的伊加自身抗体，一旦怀疑新生儿LABD，应立即停止母乳喂养。
IMPORTANCE Neonatal linear immunoglobulin A (IgA) bullous dermatosis (LABD) is a rare disease that can be fatal when associated with respiratory failure. All previously reported cases of neonatal LABD have been in newborns with healthy asymptomatic mothers, and the pathogenic IgA was of unknown origin.OBJECTIVE To clarify the origin of IgA associated with LABD in neonates born of healthy asymptomatic mothers.DESIGN, SETTING, AND PARTICIPANTS This case study analyzed the laboratory findings of a single breast-fed newborn male with neonatal LABD admitted to the Keio University Hospital in Tokyo and his healthy asymptomatic mother. The healthy newborn developed life-threatening blisters and erosions of the skin and mucous membranes on day 4 after birth. Blood serum, skin, and maternal breast milk were examined for IgA autoantibodies.MAIN OUTCOMES AND MEASURES Histopathologic and immunofluorescence analyses of specimens (serum, skin, and breast milk) from the patient and his mother.RESULTS Histopathologic evaluation of the newborn's skin revealed subepidermal blisters with neutrophil infiltrates, and immunofluorescence testing showed linear IgA deposition along the basement membrane zone (BMZ), which lead to the diagnosis of neonatal LABD. Indirect immunofluorescence using normal human skin after treatment with 1-mol/L sodium chloride showed the patient to have circulating IgA binding to the dermal side of BMZ. Immunohistochemical staining proved the deposition of secretory IgA in the neonatal skin by demonstrating the presence of J chain-not been seen in other LABD cases-indicating that the autoantibodies producing the blisters were derived from the maternal breast milk. Although no circulating IgA against the skin was detected in mother's sera, the breast milk contained IgA that reacted with the dermal side of the BMZ. No new blister formation was observed after cessation of breastfeeding.CONCLUSIONS AND RELEVANCE The results of this case study suggest a passive transfer of pathogenic IgA to a newborn from an asymptomatic mother via breast milk. In prior reports, no serum from asymptomatic mothers of newborns with LABD had IgA autoantibodies binding to skin components; however, in this case, we found that the maternal breast milk contained IgA autoantibodies associated with neonatal LABD. In neonatal LABD, maternal breast milk should be examined for IgA autoantibodies and breast milk feeding should be discontinued as soon as neonatal LABD is suspected.