Diagnosis and treatment
Diagnosis is based on physical findings in an infant aged <6 months and detection of anti-Ro/SSA and anti-La/SSB antibodies in the child or mother. It should be suspected in children with congenital heart block as it is responsible for over 85% of cases.
All infants diagnosed with neonatal lupus erythematosus or born to women with anti-Ro/SSA or anti-La/SSB antibodies should have an ECG to detect heart block. If this is abnormal, referral to a pediatric cardiologist is warranted. All pregnant women with anti-Ro/SSA or anti-La/SSB antibodies should undergo regular fetal echocardiography to detect heart block. The initiation of dexamethasone or plasmapheresis as a preventative measure against congenital heart block in high-risk pregnant women is currently under consideration but is not yet justified.3
Treatment includes photoprotection (avoiding sunlight using clothing and other protective measures) and time, as nearly all cases resolve within 6 months without scarring. Mild topical steroids may be helpful. It is important to note that children with neonatal lupus may be at higher risk of developing autoimmune disorders or rheumatic disease later in life.2
Outcome
For this patient, the skin lesions were scraped with the side of a microscope slide and KOH with DMSO was used to dissolve the epithelial cells. The preparation was negative for fungal elements.This infant had anti-Ro, anti-La, and anti-RNP levels of 128.3, 150.3, and 128, respectively. Normal range is 0 to 19 for each autoantibody. Her mother’s autoantibody levels were similarly abnormal at 138.1, 158.8, and 169.1.
The patient was referred to a pediatric cardiologist for follow-up; results of a second echocardiogram and ECG were normal. The infant was seen again at 7 weeks; cutaneous lesions were present but somewhat improved, and she was otherwise doing well. Given the high probability of lupus occurring in future pregnancies, the mother received appropriate education.
CORRESPONDENCE
Keith A. Frey, MD, Department of Family Medicine, Mayo Clinic Arizona, 13737 North 92nd Street, Scottsdale, AZ 85260. E-mail: frey.keith@mayo.edu