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 ne onatal lupus erythematosus (NLE) with regard to maternal disease, sex of ch ild, 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-UI-ribonucleoprotein antibodies and their 57 infants (20 boys and 37 girls) diagnosed with cutaneous NLE (absent hea rt disease) between 1981 and 1997. At detection of the child's rash, 13 mot hers were asymptomatic, 11 had an undifferentiated autoimmune syndrome (UAS ), 9 had systemic lupus erythematosus (SLE), 7 Sjogren's syndrome (SS), 6 S LE/SS, and I rheumatoid arthritis/SS; 20 reported photosensitivity. Within 5 years, 7 asymptomatic mothers experienced disease progression: I develope d photosensitivity, 2 SLE, 3 SS, 1 SLE/SS; in 2 mothers UAS progressed to S LE; and 2 mothers with SS developed SLE. The infant's rash often followed U V light exposure; mean age at detection was 6 weeks, and mean duration uas 17 weeks. All had facial involvement (periorbital region most common) follo wed by the scalp, trunk, extremities, neck, and intertriginous areas. In 37 , the rash resolved without sequelae, 43% of which were untreated. A quarte r had residual sequelae that included telangiectasia and dyspigmentation. O ne child developed Hashimoto's thyroiditis, and 2 developed systemic-onset juvenile rheumatoid arthritis. Of 20 subsequent births, 7 children were hea lthy, 2 had congenital heart block (CHB) only, 4 CHB and skin rash, and 7 s kin rash only. Conclusions: Future pregnancies should be monitored by serial echocardiogra ms, given the substantial risk for heart block. infected children should be observed for later development of a rheumatic disease.