Cutaneous manifestations of neonatal lupus without heart block: characteristics of mothers and children enrolled in a national registry.

OBJECTIVE: To extend the information base on cutaneous manifestations of neonatal lupus erythematosus (NLE) with regard to maternal disease, sex of child, onset, localization, influence of UV light, prognosis, and recurrence rates in subsequent pregnancies.
METHODS: Review of records from the Research Registry for Neonatal Lupus.
RESULTS: The cohort includes 47 mothers (83% white) whose sera contain anti-SSA/Ro, anti-SSB/La, and/or anti-U1-ribonucleoprotein antibodies and their 57 infants (20 boys and 37 girls) diagnosed with cutaneous NLE (absent heart disease) between 1981 and 1997. At detection of the child's rash, 13 mothers were asymptomatic, 11 had an undifferentiated autoimmune syndrome (UAS), 9 had systemic lupus erythematosus (SLE), 7 Sjögren's syndrome (SS), 6 SLE/SS, and 1 rheumatoid arthritis/SS; 20 reported photosensitivity. Within 5 years, 7 asymptomatic mothers experienced disease progression: 1 developed photosensitivity, 2 SLE, 3 SS, 1 SLE/SS; in 2 mothers UAS progressed to SLE; and 2 mothers with SS developed SLE. The infant's rash often followed UV light exposure; mean age at detection was 6 weeks, and mean duration was 17 weeks. All had facial involvement (periorbital region most common) followed by the scalp, trunk, extremities, neck, and intertriginous areas. In 37, the rash resolved without sequelae, 43% of which were untreated. A quarter had residual sequelae that included telangiectasia and dyspigmentation. One child developed Hashimoto's thyroiditis, and 2 developed systemic-onset juvenile rheumatoid arthritis. Of 20 subsequent births, 7 children were healthy, 2 had congenital heart block (CHB) only, 4 CHB and skin rash, and 7 skin rash only.
CONCLUSIONS: Future pregnancies should be monitored by serial echocardiograms, given the substantial risk for heart block. Affected children should be observed for later development of a rheumatic disease.