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

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.