Author/Authors :
Ziaee Vahid نويسنده , Kadivar Maliheh نويسنده Department of Neonatal Care Unit, Children’s Hospital Medical Center, Tehran University of Medical Sciences, Tehran, Iran; , Sangsari Razieh نويسنده Department of Neonatal Care Unit, Children’s Hospital Medical Center, Tehran University of Medical Sciences, Tehran, Iran; , Shahbaznejad Leila نويسنده Department of Pediatrics, Tehran University of Medical
Sciences, Tehran, IR Iran
Abstract :
Introduction Neonatal lupus erythematous (NLE) is a neonatal
autoimmune disease, due to passage of maternal autoantibodies against
Ro/SS-A, La/SS-B, and U1-ribonucleoprotein, with multi organ
involvement. Although the clinical manifestations of NLE include
cutaneous, cardiac, hematologic, and hepatobiliary system involvement,
the most common clinical manifestations are skin rash and heart
involvement. Congenital heart block in the absence of structural cardiac
abnormalities is the most commonly observed manifestation. The most
clinical manifestations of NLE are self-limited and resolve without
serious treatment, except heart block that may need a pacemaker. In rare
instances, more severe complications may occur, and necessitate more
aggressive immune suppressive therapy. Case Presentation Here we present
a neonate girl who developed cutaneous, cardiac, liver, brain, splenic,
kidney, and hematologic abnormalities. After 2 months of aggressive
immune suppressive therapy she could be discharged. The treatment was
tapered stepwise and discontinued at the age 1 year. At the age of 4
years she was in a favorable health condition.