Lupus eritematoso neonatal: reporte de cuatro casos

Abstract

Neonatal lupus erythematosus is a very rare disease, clinically characterized by skin lesions that resemble those of subacute or discoid lupus erythematosus and/or congenital heart block. Generally, when patients have skin manifestations, they have no cardiac defects and vice-versa; however, in 10% of cases these manifestations may coexist. Other findings may include hematologic, hepatic and neurological abnormalities. This condition is caused by the transplacental passage of maternal autoantibodies against Ro (95%), La and, less frequently, U1-ribonucleoprotein (U1-RNP). The present case report describes four patients with clinical, histopathological and immunological findings compatible with neonatal lupus erythematosus, their treatment and progress. Keywords: Cutaneous; Heart diseases; Lupus erythematosus, pediatricsResumen: El lupus eritematoso neonatal es una enfermedad poco frecuente, caracterizada clínica-mente por alteraciones cutáneas semejantes al lupus subagudo o discoide y/o bloqueo cardíaco congénito. Generalmente, cuando los pacientes presentan manifestaciones cutáneas, no tienen anormalidades cardiológicas y viceversa, aunque en un 10% de los casos ambas manifestaciones pueden coexistir. Puede acompañarse también de alteraciones hematológicas, hepáticas y neurológicas. Es causado por el pasaje trasplacentario de anticuerpos maternos anti Ro (95%), anti La y menos frecuentemente anti U1RNP. Presentamos cuatro pacientes con hallazgos clínicos, histopatológicos e inmunológicos compatibles con lupus eritematoso neonatal, su tratamiento y evolución.

Maternal Autoantibodies in Autism

“…9 While such conditions are relatively uncommon and the offending IgG are cleared from the infant’s circulation within the first 6 months of life, situations where IgG alters important developmental processes can lead to permanent defects. For example, in some cases of neonatal lupus erythematosis, 10 characteristic maternal antibodies, which bind to the ribosomal Ro ribonucleoprotein, prevent proper heart formation in the developing fetus, thereby requiring corrective surgery in the newborn.…”

Neonatal Lupus Erythematosus

“…In addition to its presence in the skin and heart, the Ro antigen is also found in the liver, bowel, lungs, brain, and blood cells—the tissues that are most often affected by NLE [3]. Ultraviolet radiation and estrogens increase Ro antigen expression on the surface of the keratinocyte [3].…”

“…Ultraviolet radiation and estrogens increase Ro antigen expression on the surface of the keratinocyte [3]. Although ultraviolet radiation can induce or exacerbate the skin lesions, it is not required for their development [10].…”

“…Neonatal lupus erythematosus (NLE) refers to a clinical spectrum of cutaneous, cardiac, and systemic abnormalities observed in newborn infants whose mothers have autoantibodies against Ro/SSA, La/SSB, and, less commonly, U1-ribonucleoprotein (U1-RNP) [1–3]. The condition was first described in 1954 by McCuistion and Schoch who reported a case of transient lupus skin lesion in an infant with an ANA-positive mother [4].…”

Scars on the skin, scars in the heart

“…[345] Both mother and the child are at an increased risk of developing some rheumatologic or autoimmune disease in future. [3567]…”