Pediatric Rheumatology Online Journal → July 2003 → Childhood Lupus→ Neonatal Lupus → Abstract #106

THE PREVALENCE, ASSOCIATED FEATURES AND OUTCOME OF CUTANEOUS NEONATAL LUPUS ERYTHEMATOSUS ^{1, 2}

C. Boros,¹ D. Spence,¹ H. Convery,¹ B. Krafchik,² E. Silverman.¹

¹Division of Rheumatology, Hospital for Sick Children, Toronto, ON, Canada

Aims To determine the prevalence of cutaneous NLE, outcome of rash and associated features in a cohort of infants followed at the HSC.
Methods The clinic records of all infants followed in the NLE clinic between November 1989 and November 2002 were reviewed for the presence of rash, its location, the presence of scarring and other associated features of NLE. Of 259 infants in the clinic, 58 (22%) had cutaneous NLE.
Results: Infants. Twenty-five (43%) had disease limited to the skin. The most common associated feature was elevated LFTs (29%) followed by hematological involvement (24%). Seven infants (12%) had CCHB and 2 (3.4%) had hydrocephalus.
Most infants had rash located on the face (n=54), trunk (n=20) and/or scalp (n=15). Five had rash all over the body. The most common age of onset was between 4-8 weeks of age (n=24) with a range of 0-12 weeks: 12 infants had rash present at birth. Of 28 infants followed to resolution of rash, 11 developed dyspigmentation, scarring, atrophy or telangiectasiae at the site(s) of previous rash (median follow-up 11m: range 2-57 m, median resolution time 6m: range 5 weeks-12m).
Results: Mothers Of 53 mothers, 51 were anti-Ro antibody positive, 36 anti-La antibody positive and 35 Rheumatoid Factor positive at the time of diagnosis. Only 10 had antibodies to DsDNA. Of 50 mothers who had health status documented, 13 were healthy at diagnosis, 20 had SLE and 10 had Sjogren's syndrome. Other diagnoses included cutaneous lupus (n=3), Raynaud's (n=3), hypothyroidism (n=2), cutaneous vasculitis (n=1) and MCTD (n=1).
Conclusion In our study, 11 of 28 infants with the rash of NLE developed sequelae at a median follow-up of 11 months. So far, no infant has developed connective tissue disease, although follow-up of these infants is ongoing (median 7m, range 0-109m). Most mothers were symptomatic at the time of NLE diagnosis and that most of the symptomatic mothers had SLE. These data may assist in identification of risk factors for adverse outcome in infants with cutaneous NLE.