Neonatal Linear Immunoglobulin A Bullous Dermatosis: A Critical Case Recovering after Prompt Recognition, Intensive Management, and Breastfeeding Interruption - A Case Report.

Neonatology Pub Date : 2024-09-13 DOI:10.1159/000540770

Dimitra Papasavva, Leila Dosso, Marie-Anne Morren, Lionel Fontao, Laura Bruschi, François Gorostidi, Thomas Ferry, Emmanuella Guenova, Céline J Fischer Fumeaux, Sébastien Joye

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Abstract

Introduction: Neonatal linear immunoglobulin A (IgA) bullous dermatosis (NLABD) is a rare, life-threatening, mucocutaneous bullous disorder. The pathogenesis and optimal treatment remain poorly defined and raise critical clinical challenges.

Case presentation: We present a case of a full-term female infant with severe cutaneous and respiratory symptoms due to NLABD. Diagnosis was confirmed by immunofluorescence on the infant's skin biopsy, while IgAs directed against the basement membrane of the skin and mucosa were identified in the mother's milk. The infant fully recovered after nearly 8 weeks of intensive multidisciplinary care, including non-invasive ventilation, nutritional support, wound care, systemic corticoid treatment, and breastfeeding discontinuation.

Conclusion: This case underscores the importance of timely adequate diagnosis and management of this rare and serious condition. Moreover, it adds novel evidence documenting the presence of pathogenic IgAs in breastmilk.

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新生儿线性免疫球蛋白 A 大疱性皮肤病：及时识别、强化治疗和中断母乳喂养后康复的危重病例 - 病例报告。

简介新生儿线性免疫球蛋白 A (IgA) 大疱性皮肤病（NLABD）是一种罕见的、危及生命的粘膜大疱性疾病。其发病机制和最佳治疗方法仍未明确，给临床带来了严峻的挑战：我们介绍了一例因 NLABD 而出现严重皮肤和呼吸道症状的足月女婴。通过对婴儿皮肤活检进行免疫荧光确诊，同时在母亲的乳汁中发现了针对皮肤和粘膜基底膜的 IgAs。经过近 8 周的多学科强化护理，包括无创通气、营养支持、伤口护理、全身皮质激素治疗和停止母乳喂养，婴儿完全康复：本病例强调了对这种罕见的严重疾病进行及时充分诊断和治疗的重要性。此外，该病例还提供了新的证据，证明母乳中存在致病性 IgAs。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Neonatology

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