A female, term newborn born to a mother with a history of idiopathic thromb
ocytopenic purpura and antinuclear antibodies, single-stranded A antibody,
and IgM anticardiolipin antibodies presented with immune thrombocytopenia,
disseminated intravascular coagulation (DIC), microangiopathic hemo lytic a
nemia, and a characteristic lupus rash in the periorbital areas. She respon
ded to combined treatment with dexamethasone and intravenous immunoglobulin
(IVIG). At age SI months, she was readmitted with severe thrombocytopenia,
DIG, and microangiopathic hemolytic anemia. She again responded to IVIG. T
his suggests that microangiopathic hemo lysis can be a presenting symptom i
n neonatal lupus erythematosus and that reoccurrence of the microangiopathi
c hemolysis may occur even after the disappearance of lupus antibodies.