Neonatal Lupus Erythematosus : Clinical Manifestations and Management

AMORNSRI CHUNHARAS, M.D.*, PRACHA NUNTNARUMIT, M.D.*, SURADEJ HONGENG, M.D.*, AMPAIWAN CHAUNSUMRIT, M.D.*

Affiliation : *Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.

AbstractThe authors report 6 cases of neonatal lupus erythematosus (NLE) who were seen at Rama thibodi Hospital from 1993 to 2000. The female to male ratio was 1 : 5. Cutaneous lesions were the major manifestation in all cases. Other clinical manifestations were thrombocytopenia, hepatospleno megaly and mild elevation of liver enzymes. Skin rashes mostly erupted at 3-6 weeks old. None had a complete heart block but one had abnormal electrocardiograph (ECG) changes compatible with Wolff-Parkinson-White syndrome (WPW). Four of six patients had thrombocytopenia. All of the abnormalities resolved spontaneously except thrombocytopenia. Three of six needed blood transfusion to replace blood loss from gastrointestinal bleeding. Intravenous immunoglobulin (IVIG) 2 g/kg was given in 3 cases with good response in two of three cases. Platelets rose rapidly and maintained at a normal level within 24-48 hours. Combined therapy with corticosteroid 2 mg/kg was given to 1 case with good outcome. Telangiectasia was the most common sequelae especially in patients who had periorbital lesions resembling raccoon's eyes. The authors conclude that IVIG in the dose of 1g/kg for 1-2 days is an effective treatment for NLE with severe thrombocytopenia especially when cortico steroid is contraindicated.

Keywords : Neonatal Lupus Syndrome, NLE, Thrombocytopenia, Cutis Marmorata, IVIG

All Articles Download

Information

Related Links