Systemic Lupus Erythematosus With Libman-Sachs Endocarditis in a 9-Month-Old Infant With Neonatal Lupus Erythematosus and Congenital Heart Block

Since the first report of a patient with a congenital discoid lupus erythematosus rash in 1954 by McCuistion and Schoch, multiple instances of neonatal lupus erythematosus have been described. It is now a well-described syndrome of congenital heart block, transient rash, or both, associated with transplacental passage of antibodies to Sjogren's syndrome A (Ro/SSA) from mothers with overt or latent connective tissue disease. The syndrome usually resolves when the infant is 6 months of age. Neonatal lupus erythematosus without congenital heart block is thus thought to be a transient, relatively benign, phenomenon. Even with congenital heart block, the prognosis can still be good, especially in the absence of structural cardiac abnormality.