Early cholestasis in neonatal lupus erythematosus

Neonatal lupus erythematosus is an immune-mediated disease caused by transplacental passage of maternal autoantibodies, primarily anti-Ro (SSA) and anti-La (SSB). The major clinical manifestations are congenital heart block, cutaneous lupus lesions, and hematologic problems. Hepatic, pulmonary, and neurological involvements are rare. We report a 5-day-old male neonate, born to a clinically asymptomatic mother, presenting with conjugated hyperbilirubinemia, cutaneous lupus lesions, congenital heart block, and thrombocytopenia. Both the neonate and his mother had high titers of antinuclear antibodies (1:640), anti-Ro (SSA), and anti-La (SSB) antibodies. The thrombocytopenia improved with prednisolone (2 mg/kg/day) for 14 days. The skin lupus rashes and bilirubin resolved 2 months later, and liver enzymes were completely normal by 6 months.