Neonatal lupus erythematosus (NLE) is associated with cutaneous lesions, congenital heart block (CHB), hepatic disease and thrombocytopenia. IgG antibodies to Ro and/or La cross the placenta and participate in the development of the clinical manifestations. Mothers of babies with NLE are likely to develop collagen vascular diseases with time. Infants with NLE are at risk to develop other autoimmune diseases during childhood or adolescence.
NLE appears to occur in about 1 in 20,000 live births and can affect all ethnic groups. Females are affected by NLE preferentially 2:1 for cutaneous lupus, but males and females are equally affected with congenital heart block (CHB); 70% to 80% of babies with CHB had NLE.
One percent of NLE patients develop hepatic disease. Thrombocytopenia or neutropenia is observed in about 10% of cases. Both usually resolve within weeks of birth.