Liver Involvement in Neonatal Lupus Erythematosus: The Spectrum of Disease. 489

Neonatal lupus erythematosus (NLE) is classically characterized by complete congenital heart block, the presence of cutaneous lupus lesions, or both. Hepatic involvement has recently been recognized as a component of this syndrome. The described findings include cholestasis, lymphocytic infiltration and ductular hyperplasia. We present 2 patients with heretofore undescribed hepatic lesions associated with NLE. Patient 1 presented with neonatal jaundice, a facial rash, and complete heart block. Percutaneous liver biopsy done at age 1 month revealed hemosiderosis. A repeat biopsy one month later revealed a paucity of intra-hepatic bile ducts. The patient died at age 19 months. On autopsy, there was presence of bridging fibrosis and focal nodular formation. Patient 2 presented with jaundice, respiratory distress, hypoglycemia, and complete heart block. Liver biopsy done at age 6 weeks revealed large fat droplets within the hepatocytes in a panlobular distribution. Further evaluation and a repeat liver biopsy ultimately led to a diagnosis of glycogen storage disease type Ib. The hepatic pathology in NLE is probably more heterogeneous than previously believed. The presenting symptom of children with liver disease and associated NLE is usually jaundice. Nevertheless, the precise nature of the hepatic lesion cannot be predicted. In patients with NLE and jaundice, the need for biopsy diagnosis of the hepatic lesion is critical for appropriate diagnosis and management.