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Neonatal lupus syndrome (NLS) is a rare immune-mediated condition that results from the passive transfer of maternal autoantibodies to the fetus. Autoantibodies known to cause NLS include Sjogren (anti-SSA and anti-SSB) and anti-U1-ribonucleoprotein immunoglobulin G (IgG) antibodies. These antibodies are typically seen in association with maternal systemic lupus erythematosus (SLE), Sjogren's syndrome, rheumatoid arthritis, and other systemic autoimmune conditions. Fetuses and newborns born to women with known rheumatologic disease and seropositivity for high-risk antibodies are monitored closely for clinical manifestations of NLS. However, a subset of newborns diagnosed are born to women with no previous diagnosis of autoimmune disease. Here, we report a case of a newborn who presented with a leading to the diagnosis of both infantile and maternal autoimmune disease.

A 2-week-old male born at 36 weeks presented for a routine well-child exam with maternal concern about a facial rash that had been present since approximately one week of life.  On examination, he had multiple annular, raised erythematous patches with central clearing on his face.      Over the following weeks, these lesions gradually expanded and coalesced, and new lesions appeared on his face, limbs, and trunk. His mother underwent laboratory evaluation revealing positive ANA at 1:1280 titer, as well as positive anti-SSA and SSB antibodies, confirming the diagnosis of NLS. Further evaluation of the infant with EKG and echocardiogram did not reveal any evidence of congenital heart block or other cardiac manifestations of NLS. His rash resolved spontaneously by six months of age.

Neonates presenting with a classic rash or congenital heart block should be evaluated for neonatal lupus syndrome. The absence of known maternal rheumatologic disease does not preclude a diagnosis of neonatal lupus syndrome and mothers of these infants should be monitored closely for development of rheumatologic disease.

Publication Date

5-8-2026

Disciplines

Pediatrics

Comments

2026 Research Day Corewell Health West, Grand Rapids, MI, May 8, 2026. Abstract 1969

More Than Just a Rash: A Case of Neonatal Lupus Syndrome Uncovering Maternal Autoimmunity

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