Catalina MontanéHojman, LiaHasbún, María Trinidad2024-06-062024-06-062023Montané Catalina, Hojman Lía, Hasbún Trinidad. Neonatal lupus erythematosus, a clinical case. Andes pediatr. [Internet]. 2023 Apr [cited 2024 June 06] ; 94( 2 ): 240-245. Available from: http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S2452-60532023000200240&lng=en. http://dx.doi.org/10.32641/andespediatr.v94i2.3889.https://hdl.handle.net/11447/9065Neonatal lupus erythematosus (NLE) is a very rare autoimmune disease, occurring in neonates born to mothers who present auto-antibodies to cytoplasmic antigens of Sjögren’s syndrome. In most cases, the clinical course is benign toward spontaneous resolution, but there is a group of patients who develop severe involvement of the cardiac conduction system, therefore, early detection is critical. Objective: To describe a clinical case of neonatal lupus erythematosus emphasizing the importance of timely diagnosis in the patient and the mother. Clinical Case: A 33-year-old woman, with a history of hypertension, came to the dermatology department for her 15-day-old male neonate who presented a recent onset of round, erythematous, raised-edged, and non-scaling plaques consistent with NLE. Cardiac conduction involvement was ruled out. Newborn’s laboratory tests showed moderate neutropenia, mild increase of transaminases, and positive anti-Ro and anti-La antibodies. On directed anamnesis, the mother reported a personal history of symptoms consistent with connective tissue disease, such as fatigue, alopecia, and xerophthalmia. Antinuclear antibodies from the mother showed titers of 1/1280 in a speckled pattern, positive anti-double-stranded DNA antibodies, and anti-Ro and Anti-La antibodies. Schirmer Test was consistent with dry eye, therefore, Systemic Lupus Erythematosus associated with Sjögren’s Syndrome was diagnosed. The infant was followed up for 5 months with remission of cutaneous manifestations and normalization of laboratory tests. Conclusion: Although cutaneous manifestations of NLE are benign and transient in the newborn, these can be associated with other life-threatening manifestations that require an active search and prompt management by the medical team. A 25% of mothers of newborns with NLE are asymptomatic or unaware of their SLE diagnosis before delivery, so timely diagnosis of NLE leads to the diagnosis of asymptomatic mothers, improving their follow-up and treatment.6 p.enAtribución-NoComercial-CompartirIgual 3.0 Chile (CC BY-NC-SA 3.0 CL)Neonatal Lupus ErythematosusNewbornSjögren’s syndromeConnective Tissue DiseaseArticlehttp://dx.doi.org/10.32641/andespediatr.v94i2.3889