A baby with red plaques on the face and a first-degree heart
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A 3 month old boy was brought to the clinic who had developed several round red lesions on his face. The problem had begun 3 weeks post partum. The baby had been born at full term following an uncomplicated pregnancy. The patient’s mother was fit and well with no medical history of note-specifically she did not report any previous infections, or dermatological or autoimmune diseases.

The baby had been seen when he was 10 weeks old and given topical antifungal treatment—miconazole cream—and a low potency topical corticosteroid cream—hydrocortisone—which had no effect after a 2-week trial. On examination six well defined, erythematous annular plaques, with indurated borders, on the baby’s face were found. Laboratory blood tests showed that both the mother and child were positive for antibodies against extractable nuclear antigen. They were also positive for anti-Sjogren’s syndrome type A (SSA) and anti-Sjogren’s syndrome type B (SSB) antibodies.

Taken together with the findings of the biopsy, neonatal lupus erythematosus was diagnosed. Given that heart abnormalities are associated with the condition, paediatric cardiologist requested an ECG which showed findings consistent with first-degree atrioventricular block. 3 months later at a follow-up appointment, all the baby’s skin lesions had resolved without any scarring. Finally, again given the diagnosis and the positive antinuclear antibodies, the patient’s mother was referred to a rheumatologist who diagnosed Sjogren’s syndrome.

Neonatal lupus erythematosus is a passively acquired, self-limiting, autoimmune disease thought to be caused by the transplacental passage of anti-SSA and anti-SSB antibodies from mother to fetus: the skin—and more importantly—the heart are particularly affected.

The cutaneous manifestations in the baby usually develop within the first few weeks of life—typically appearing on the scalp and periorbital areas. The association of the skin lesions with positive anti-SSA and anti-SSB tests in both mother and baby is well recognised, and a skin biopsy is not necessarily required to make the diagnosis. The important complication of congenital heart block- which usually develops in utero and generally resolves- is rare and found in approximately 2% of pregnant women with anti-SSA or anti-SSB antibodies. The skin manifestations in neonatal lupus spontaneously resolve after 4-6 months.

Source: https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(20)32176-0/fulltext?rss=yes#rele1237f8f-fba8-421f-b6e0-cb008ca30e95