SAN ANTONIO — All cases of congenital heart block are caused by neonatal lupus, Dr. Bernice Krafchik said at a meeting sponsored by Skin Disease Education Foundation.
About half of neonatal lupus babies will have congenital heart block. Part of the challenge in diagnosis is that most often the mothers are asymptomatic, added Dr. Krafchik, professor emeritus of pediatrics and medicine, the Hospital for Sick Children, University of Toronto.
The heart block is usually complete heart block. “These patients often require a pacemaker—you have to really watch these kids closely; the pacemaker itself can have adverse events and cause death.”
In general, babies with just cutaneous lesions from neonatal lupus erythematosus respond well to therapy, according to Dr. Krafchik.
“Skin patients really do very well and the lesions disappear. But they may develop systemic lupus erythematosus [SLE] later.” Babies with neonatal lupus skin lesions usually are born to mothers with SLE, which she said is one of the new facts emerging about neonatal lupus.
About 50% of babies born with neonatal lupus have skin lesions. There are four presentations: periorbital lesions that produce a “raccoon” face, annular erythema with atrophy, central erythema with an edge, and telangiectasias.
The telangiectasias are less common but might persist into adulthood. Some telangiectasias respond to laser treatment, although scarring can be problematic, said Dr. Krafchik.
Neonatal lupus can cause thrombocytopenia and hepatitis as well. Hepatitis occurs in approximately 10% of affected neonates and is usually mild with “excellent recovery,” she said at the meeting.
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