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.
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