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A Journal on Pediatrics, Neonatology, Adolescent Medicine,
Child and Adolescent Psychiatry

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Minerva Pediatrica 2012 April;64(2):251-6

language: Italian

A case of neonatal lupus syndrome and congenital atrioventricular block associated with maternal antibodies antiro/ss-a

De Leonibus C. 1, Lembo C. 1, Giliberti P. 1, Rojo S. 1, Foglia M. C. 2, Giordano L. 1, Fratta A. 1

1 Divisione di Neonatologia e Terapia Intensiva Neonatale, A.O.R.N. “V. Monaldi”, Napoli, Italia;
2 Laboratorio di Patologia Clinica e Microbiologia, A.O.R.N. “A. Cardarelli”, Napoli, Italia


The neonatal lupus erythematosus syndrome (LEN) is a disease due to the transplacental passage of maternal antiextractable nuclear antigens (ENA) antibodies, particularly anti-Ro/SS-A and anti-La/SS-B. The disease affects neonates born from mothers with autoimmune diseases. It is characterized by erythematous annular polycylic skin lesions, slightly scaling with prevalent face localization, hematologic and liver diseases and only in 2% of cases with extracutaneous lesions including complete atrioventricular block. The Authors describe a case of LEN characterized by isolated atrioventricular block at birth and endocardial fibroelastosis without skin lesions in a preterm infant female. She was born from asymptomatic, ANA (Anti-Nuclear Antibodies) and ENA (anti-Extractable Nuclear Antigen) positive mother, with a previous miscarriage at the 5th week of gestation.

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