Neonatal lupus erythematosus (NLE) is a syndrome of cutaneous lupus or
congenital heart block or both and/or systemic manifestations which a
ppears in children of women with connective tissue diseases (SLE, Sjog
ren Syndrome, etc.) or asymptomatic mothers with antibodies anti-Ro/SS
A, anti-La/SSB and, rarely, anti-U1 RNP. The management of this syndro
me, for its particular features, involves various different health pro
fessionals such as rheumatologist, gynecologist, neonatologist and ped
iatric cardiologist. In this paper we present an update on NLE as a co
ntribution for the better understanding of this condition and for the
subsequent preventive and therapeutical choices.