Andres Bayona, MD, Robert M Lapus, MD
Department of Emergency Medicine, University of Texas Medical School at Houston, Houston, Texas
Children’s Memorial Hermann Hospital, Houston, Texas
A one month old girl with a history of congenital complete Atrioventricular (AV) block with an implanted pacemaker placement on day of life 6, presented to the Emergency Department (ED) with rash and fever. One week prior to presentation she developed a rash on her face, which slowly spread to her chest, abdomen and extremities. On the day of presentation she developed a fever at home. Mom brought her to the pediatrician, who then referred them to the ED.
In the ED, her initial temperature was 101.4 ˚F, pulse 100 (paced) beats per minute, respiratory rate 36 breaths per minute and blood pressure 86/57 mmHg. Physical exam showed a healed surgical site and annular erythematous plaques, some with scaling, and some with central pallor, shown below:
Most likely the fever and rash were unrelated. Given the infant’s age, she was worked up, started on antibiotics, and admitted. The differential diagnosis for erythematous annular plaques is broad and includes infection vasculitis, erythema multiforme, and autoimmune disease. Given the past medical history of congenital complete AV block, the rashes were identified as cutaneous lesions of neonatal lupus erythematosus (NLE). Antibodies to Ro and La were sent and returned positive. The lesions were eventually treated with topical corticosteroids. Mom had no rheumatologic symptoms at the time of delivery.
About 70% of infants with NLE have cutaneous findings, two-thirds of those will present at birth and the rest can present between 2-5 months of life. Patients with NLE may present to the ED due to skin lesions, however, NLE can affect the cardiac, hepatic, and hematologic systems as well. Maternal history is important, but more than half the mothers at the time of child birth are asymptomatic for Lupus or other collagen vascular diseases.