Neonatal lupus presenting as a non-specific rash in primary care

Neonatal lupus erythematosus is a rare autoimmune disease caused by passive transplacental acquisition of maternal autoantibodies manifesting in cutaneous, cardiac, haematological and hepatobiliary abnormalities. The hallmark dermatological finding is erythematous annular lesions with a predilection for photo-exposed areas of the skin. We present a case of a female infant born to a mother with Sjogren's syndrome, who initially presented to an ambulatory care setting with non-specific erythematous papules involving the face and scalp. Within 6 days the rash changed in appearance, consisting of widespread erythematous annular and polycyclic lesions with central violaceous clearing and atrophy. Serological tests revealed asymptomatic anemia and leukopenia, elevated liver enzymes, and positive antinuclear antibodies (ANA) and anti-SSb/La antibodies. Further cardiac evaluation was normal. She was managed conservatively in the outpatient setting with topical steroids, avoidance of sunlight and fluorescent light exposure, and primary care, rheumatological and dermatological follow-up.