We report a newborn infant girl, born to consanguineous parents, with recurrent intracranial hemorrhage secondary to congenital factor V deficiency with factor V inhibitor. Repeated transfusions of fresh-frozen plasma (FFP) and platelet concentrates, administrations of immunosuppressive therapy (prednisolone and cyclophosphamide), and intravenous immunoglobulin failed to normalize the coagulation profiles. Exchange transfusion followed-up by administrations of activated prothrombin complex and transfusions of FFP and platelet concentrates caused a temporary normalization of coagulation profile, enabling an insertion of ventriculoperitoneal (VP) shunt for progressive hydrocephalus. The treatment was complicated by thrombosis of left brachial artery and ischemia of left middle finger. The child finally died from another episode of intracranial hemorrhage 10 days after insertion of the VP shunt.

Neonatal lupus erythematous (NLE) is a rare autoimmune mediated disease that is associated with transplacental autoantibodies. It is characterised by cutaneous manifestations and may involve cardiac, haematologic, neurologic and hepatic abnormalities. We describe two cases with clinical, pathological and immunological findings compatible with neonatal lupus erythematosus, treatment and progress. Both their mothers were asymptomatic but were found to have autoantibodies. In view of the possible serious complications such as complete heart block, a high index of suspicion coupled with a thorough evaluation of both child and mother is required.