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Case ReportOpen Access
Neonatal Lupus Erythematosus : Clinical Manifestations and Management
Chunharas A 
,
Nuntnarumit P ,
Hongeng S ,
Chaunsumrit A
,
Nuntnarumit P ,
Hongeng S ,
Chaunsumrit A The authors report 6 cases of neonatal lupus erythematosus (NLE) who were seen at Ramathibodi
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, hepatosplenomegaly
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 corticosteroid
is contraindicated.
Key word : Neonatal Lupus Syndrome, NLE, Thrombocytopenia, Cutis Marmorata, IVIG
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, hepatosplenomegaly
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 corticosteroid
is contraindicated.
Key word : Neonatal Lupus Syndrome, NLE, Thrombocytopenia, Cutis Marmorata, IVIG
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