A case of neonatal lupus erythematosus with a typical malar rash

Sir, Neonatal lupus erythematosus (NLE) is a distinct disorder that exclusively affects developing fetuses and neonates who acquire transplacental anti-SSA/Ro and/or anti-SSB/La antibodies from their mothers. The most common manifestations include transient rashes, thrombocytopenia, haemolytic anaemia and permanent congenital heart block (CHB). Although a rash is present in 15–20% of children with NLE, what differs from the adult patients is it is rarely in a malar distribution [1, 2]. Here we present a case of NLE with a typical malar rash. A newborn girl was delivered vaginally at 39 gestational weeks with a typical malar rash on her face. The rash appears butterfly-shaped across the nose, cheeks and eyelids, with a rough texture that is mostly flat, although in some areas it is slightly raised with some scattered red papules (Fig. 1a). Her mother was a 28-year-old primipara with no associated pregnancy complications. She was asymptomatic after thorough review for possible autoimmune diseases and sexually transmitted diseases (Treponema pallidum antibody negative). Her family history was also negative for autoimmune diseases. Further tests were done on the fourth post-partum day. Using the IIF technique, the mother was found to be ANA positive, with a titre of 1:>320 a homogeneous granulosus pattern, anti-Ro60 (+++), anti-Ro52 (+), anti-La (++) and an IgG level of 1090 mg/dl. The same findings were observed in the baby with exception of negative anti-La antibodies and an IgG level of 870 mg/dl. After relevant tests and careful evaluation from a multidisciplinary panel including rheumatologists, paediatricians and dermatologists, the infant showed no heart block and normal haematological, liver and renal function. The baby was discharged 7 days after birth when the rash slightly faded. After 6 months a follow-up examination showed the rash had disappeared spontaneously, with only a slight remnant in the previous location (Fig. 1b). A magnified image with dermascopy of her right cheek showed the previous rash was fading with scales and engorged microvasculature (Fig. 1c). Her blood antibodies were all negative, consistent with improvement of the skin lesion, and her IgG level had decreased to 255 mg/dl. Images of the baby on the first day post-partum and at the 6-month follow-up. (a) A baby girl with malar rash on the face at birth. The rash is butterfly-shaped across the nose, cheeks and eyelids, with a rough texture that is mostly flat, although in some areas it is slightly raised with some scattered red papules. (b) The malar rash had almost disappeared at her 6-month follow-up, with only a slight remnant in the previous location. (c) The magnified image shows the previous lesion is fading with scales and engorged microvasculature. What is fortunate for the baby is that she does not suffer from other system injuries. The negative anti-La antibodies in the baby might be one of the reasons. From Breda’s review, in the presence of both anti-SSB/La and anti-SSA/Ro antibodies, the rate of CHB increases to 5% while the rate is 2% when presenting with anti-SSA/Ro antibodies [3]. In addition, ethnicity might provide another explanation. In China and Japan, only 24% and 15%, respectively, of infants with NLE present with heart injuries [4, 5]. The incidence differs from that in Caucasians, which is >50% [6]. The baby and the mother are still under close follow-up for the potential risk of developing autoimmune diseases in the future [7, 8]. In this baby with neonatal lupus erythematosus, a malar rash on the face disappeared spontaneously. We thank Nkungu Daniel for his help with language polishing. Disclosure statement: The authors have declared no conflicts of interest.

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