Lupus eritematoso neonatal en un niño nacido de una madre con S. Sjögren.

R Manthorpe1, Å Svensson2 and L E Wirestrand3

1 Sjögren’s Syndrome Research Centre in Malmö, Jägersrovägen 80, SE-212 37 Malmö, Sweden
2 Sjögren’s Syndrome Research Centre, Department of Dermatology, UMAS (Malmö University Hospital), SE-205 02 Malmö, Sweden 3 Sjögren’s Syndrome Research Centre, Department of Dermatology, Kristianstad Central Hospital, SE-291 85 Kristianstad, Sweden

ABSTRACT

Background:
The neonatal lupus syndrome can be present as congenital heart block (CHB) or as neonatal lupus erythematosus (NLE), both seldom passively acquired autoimmune diseases. CHB starts around week 20 of pregnancy and is a lifelong event, whereas NLE is self limiting and usually starts at the 6th week of the child’s age—the maximum recorded up to week 20.

Case report:
An asymptomatic mother with primary Sjögren’s syndrome and anti-SSA/Ro52, anti-SSA/Ro60, and anti-SSB/La autoantibodies is described who, at gestational week 23 during her first pregnancy, was diagnosed as having a male fetus with CHB due to third degree atrioventricular block. The boy from the second pregnancy developed skin eruptions which clinically and by biopsy were compatible with NLE at week 20+1 post partum.

Conclusions:
Our case of NLE, starting at week 20+1 of age, seems to be the latest reported clinical case of NLE. Development of CHB and NLE in two consecutive boy pregnancies is unusual.