TY - JOUR
T1 - Neonatal Linear Immunoglobulin A Bullous Dermatosis
T2 - A Critical Case Recovering after Prompt Recognition, Intensive Management, and Breastfeeding Interruption - A Case Report
AU - Papasavva, Dimitra
AU - Dosso, Leila
AU - Morren, Marie-Anne
AU - Fontao, Lionel
AU - Bruschi, Laura
AU - Gorostidi, François
AU - Ferry, Thomas
AU - Guenova, Emmanuella
AU - Fischer Fumeaux, Céline J
AU - Joye, Sébastien
N1 - © 2024 The Author(s). Published by S. Karger AG, Basel.
PY - 2025/2/1
Y1 - 2025/2/1
N2 - 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.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.
AB - 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.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.
KW - Humans
KW - Female
KW - Infant, Newborn
KW - Breast Feeding
KW - Linear IgA Bullous Dermatosis/diagnosis
KW - Milk, Human/immunology
KW - Immunoglobulin A/analysis
KW - Skin/pathology
KW - Treatment Outcome
KW - Biopsy
UR - https://www.scopus.com/pages/publications/85205499464
U2 - 10.1159/000540770
DO - 10.1159/000540770
M3 - Article
C2 - 39278203
SN - 1661-7800
VL - 122
SP - 122
EP - 125
JO - Neonatology
JF - Neonatology
IS - 1
ER -